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Year 4 M&M > Rheumatology > Flashcards

Rheumatology Flashcards

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1
Q

What are the joint pathologies dealth with in rheumatology?

A
  • Rheumatoid arthritis
  • Gout
  • Pseudogout
  • Ankylosing spondylitis
  • Psoriatic arthritis
  • Reactive arthritis
  • IBD related arthropathy
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2
Q

What are the connective tissue diseases dealth with in rheumatology?

A
  • SLE/APS
  • Scleroderma
  • Myositis
  • Sjogrens syndrome
  • Fibromyalgia
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3
Q

What are the small vessel vasculitis dealth with in rheumatology?

A
  • GPA
  • EGPA
  • Microscopic polyangitis
  • Drug induced vasculitis
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4
Q

What are the medium vessel vasculitis dealth with in rheumatology?

A
  • Polyarteritis Nodosa
  • Kawasaki disease
  • Behcets disease
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5
Q

What are the large vessel vasculitis dealth with in rheumatology?

A
  • GCA
  • PMR
  • Takayashu’s arteritis
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6
Q

What signs can be seen in this example of a hand?

A

Rheumatoid arthritis

  • Ulnar deviation
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7
Q

What signs can be seen in this example of a hand?

A

Rheumatoid arthritis

  • Bouchards nodes (Proximal)
  • Herbedens nodes (Distal)
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8
Q

What signs can be seen in this image?

A

Psoriatic nail changes

  • Nail pitting
  • Onycholysis - Broken nails
  • Subungal hyperkeratosis
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9
Q

What signs can be seen in this image?

A

Gout

  • Gouty tophi
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10
Q

The following is an x-ray of a patient with a rheumatological condition. What can you see? What are the signs? What is the condition?

A

Rheumatoid arthritis

  • soft tissue swelling
  • Bone erosion
  • Bone displacement
  • Loss of joint space
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11
Q

What is Fibromyalgia?

A
  • Chronic pain disorder
  • Cause not known - possibly due to genetics & abnormalities in stress response system
  • Peripheral and central hyperexcitability + altered pain perception
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12
Q

Who commonly gets Fibromyalgia?

A
  • Women
  • Age 20-50yrs
  • Linked to failing to complete eduction, low income, divorce
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13
Q

What are the classic symptoms/signs from a person with Fibromyalgia?

A
  • Widespread pain at multiple sites
    • Low back pain
  • Associated with fatigue, relieved by rest
  • Morning stiffness generally
  • Headaches
  • Anxiety & depression
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14
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 38-year-old woman sees her physician with 4 years of widespread body pain. The pain began after a motor vehicle accident and was initially limited to her neck. Gradually, the pain has spread and she now complains of hurting all over, all the time. She does not have any joint swelling or systemic symptoms. She does not sleep well and has fatigue. She has irritable bowel syndrome but is otherwise healthy. Physical examination reveals a well-appearing woman with normal musculoskeletal examination, except for the presence of tenderness in 12 out of 18 fibromyalgia tender points. Routine laboratory testing is normal.

A
  • Fibromyalgia
  • Chronic fatigue syndrome
  • Hypothyroidism
  • Vitamin D deficiency
  • PMR
  • Polymyositis
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15
Q

You suspect a patient may have fibromyalgia, what investigations will you carry out?

A
  • Clinical diagnosis - Meets criteria:
    • Widespread bilateral pain - above + below waist
    • Includes axial skeleton
    • Present for >3months
    • Presence of 11 tender sites
  • Rule out other causes:
    • FBC - Normal
    • ESR/CRP - Normal
    • TFTs - Normal
    • RF - Normal
    • Vitamin D - Normal
    • ANA - Normal
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16
Q

How is Fibromyalgia managed?

A
  • No cure - aim is to reduce physical & mental symptoms, increase function
  • MDT management
  • Exercise programmes
  • CBT
  • Paracetamol/weak opioids
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17
Q

What is Sjogrens syndrome?

A

Autoimmune conditon

Lymphocyte infiltration of the exocrine glands leading to:

  • Dry eyes (Keratoconjunctivitis sicca)
  • Dry mouth (Xerostomia)
  • Enlargement of the parotid glands
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18
Q

What are the classical symptoms of Sjogrens syndrome?

A
  1. Dry eyes (Keratoconjunctivitis sicca)
  2. Dry mouth (Xerostomia)
  3. Enlargement of the parotid glands
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19
Q

How is Sjogrens syndrome classified?

A
  • Primary – Occurs by itself in isolation
  • Secondary – Occurs with other autoimmune condition
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20
Q

Who typically gets Sjogrens syndrome?

A
  • Women
  • Age 30-40yrs old
  • HLA-DR8 positive
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21
Q

What is the classic presentation (Symptoms/signs) of Sjogrens syndrome?

A
  • Dry eyes/Dry mouth (Dry mucous membranes)
  • Recurrent parotitis (Inflammation of parotid glands)
  • Dryness of trachea mucosa - dry cough
  • Fatigue
  • Blepharitis - Edges of eyelids inflammed
  • May have signs of other autoimmune conditions - if secondary
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22
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnoses?

A 45-year-old woman presents with fatigue and a history of positive ANAs. She has had recurrent sensation of sand/gravel in eyes and dry mouth every day for more than 3 months.

A
  • Sjogrens syndrome
  • Amyloidosis
  • SLE
  • Rheumatoid arthritis
  • Chronic pancreatitis
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23
Q

A patient presents with the following Hx, and you suspect Sjogrens syndrome. What investigations should be carried out?

A 45-year-old woman presents with fatigue and a history of positive ANAs. She has had recurrent sensation of sand/gravel in eyes and dry mouth every day for more than 3 months.

A
  • FBC – Normal or Anaemia of chronic disease (Normocytic anaemia)
  • ESR – Normal or Increased
  • Rheumatoid factor – Positive
  • ANA – Positive
  • Ro Autoantigen – Positive
  • Anti La autoantibodies – Positive
  • Schirmer test - filter paper on lower eyelid
  • Salivary gland biopsy
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24
Q

A patient is confirmed to have Sjogrens syndrome, how is this treated?

A

Symptomatic treatment

  • Artificial tears
  • Artificial saliva
  • Vaginal lubricants
  • Emollients for dry skin
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25
Q

What is Wegeners Granulomatosis (GPA)?

A

Granulomatosis w/polyangiitis

Vasculitis due to autoimmune inflammatory process affecting:

  • Endothelial cells
  • Small/Medium vessels + arterioles

Due to genetic + environmental factors

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26
Q

What does GPA typically affect?

A
  • Ear, Nose, Throat
  • Lungs
  • Kidneys
  • Can also affect - Joints/Sinuses/Eyes/Skin
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27
Q

How does GPA cause the symptoms it does?

A
  • Frank granuloma development ± necrotising inflammation
  • Tissues show little/no immune deposits
  • In kidney – Pauci-immune glomerulonephritis
  • T cells involved + B cell proliferation
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28
Q

Who typically gets GPA?

A
  • Males, but incidence is nearly same in females
  • Peak age 30-50yrs
  • FHx
  • Previous Parvovirus or Staph Aureus infection
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29
Q

What is the classifcal presentation (Symptoms/Signs) of GPA?

A
  • Non-specific symptoms
  • Fatigue/Malaise
  • Fever/Night sweats
  • Anorexia/Weight loss
  • Bloody Rhinorrhea - bloody Nasal discharge
  • Sinusitis
  • Facial pain
  • Nose - Ulcers/Sores/Crusting around nose
  • Joint pain
  • Cough/Dyspnoea/Wheezing
  • Respiratory signs – Haemoptysis/Stridor/Dsypnoea
  • Haematuria
  • Rashes – Small red/purple raised lesions
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30
Q

Which body systems are classically affected in GPA?

A
  • Nose - Bloody rhinorrhea
  • Skin - Rashes
  • Respiratory system - Cough/dyspnoea
  • Kidneys - Haematuria
  • Eyes - Inflammation
  • Nerves - Mononeuritis multiplex
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31
Q

A patient presents with the following symptoms, what is the most likely diagnosis? What are the differentials?

A previously healthy 61-year-old woman presents with a 3-month history of sinusitis and nasal drainage. She has noted only marginal, temporary improvement despite multiple courses of antibiotics. The nasal drainage is purulent and frequently haemorrhagic. She also has a 2-week history of migratory joint pain, mainly affecting wrists, knees, and ankles. She does not describe joint swelling. She reports having less energy and has lost 10 pounds in weight over the past 2 months. She has no respiratory, urinary, neurological, or other symptoms. Bleeding and inflammation of the nasal mucosa is noted, along with tenderness to percussion over both maxillary sinuses. The remainder of the physical examination is unremarkable. In-office urinalysis reveals 3+ microscopic haematuria and 2+ proteinuria.

A
  • GPA/Wegeners Granulomatosis
  • Anti-GBM antibody disease
  • Mixed connective tissue disease
  • SLE
  • Churg-Strauss syndrome
  • PAN
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32
Q

A patient presents with the following Hx, you suspect GPA. What investigations do you carry out?

A previously healthy 61-year-old woman presents with a 3-month history of sinusitis and nasal drainage. She has noted only marginal, temporary improvement despite multiple courses of antibiotics. The nasal drainage is purulent and frequently haemorrhagic. She also has a 2-week history of migratory joint pain, mainly affecting wrists, knees, and ankles. She does not describe joint swelling. She reports having less energy and has lost 10 pounds in weight over the past 2 months. She has no respiratory, urinary, neurological, or other symptoms. Bleeding and inflammation of the nasal mucosa is noted, along with tenderness to percussion over both maxillary sinuses. The remainder of the physical examination is unremarkable. In-office urinalysis reveals 3+ microscopic haematuria and 2+ proteinuria.

A
  • FBC - Anaemia
  • ESR - Increased
  • U&E - Increased Creatinine
  • ANCA (p&c) – Positive
  • Urinalysis – Haematuria/Proteinuria/RBC casts
  • Nasal endoscopy – Subglottic stenosis/Nasal crusting/inflammation
  • Lung function tests – Decreased if lung infiltrates
  • CXR – May have cavity formation + infiltrates
  • Biopsy of affected tissue (Nasal mucosa/Lung/Kidney) – Vasculitis & granuloma (Minimal/absent immune deposits)
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33
Q

How is a patient with GPA managed?

A
  • Asymptomatic w/no organ damage – May not need immunosuppression

Relapse/Active

  • Methotrexate to induce remission
  • or Mycophenelate mofetil

If life-threatening:

  • Cyclophosphamide or Rituximab
  • Prednisolone
  • Plasma exchange
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34
Q

What complications can arise from GPA?

A
  • AKI
  • Respiratory failure
  • Chronic conjunctivitis
  • Nasal septum perforation
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35
Q

What is Churg-Strauss syndrome?

A
  • Eosinophilic granulomatosis with Polyangiitis (GPA)
  • Diffuse autoimmune vasculitis affecting small/medium sized arteries
  • Associated with asthma
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36
Q

Which body systems are affected by EGPA?

A
  • Coronary vessels
  • Cerebral vessels
  • Respiratory system - Asthma
  • GIT – Bowel ischaemia/Perforation
  • Kidneys – HTN/Glomerulonephritis/CKD
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37
Q

What are the Diagnostic criteria for EGPA?

A

>4 symptoms – EGPA diagnosis

  • Asthma (Wheezing/Expiratory Rhonchi)
  • Eosinophilia >10%
  • Histological confirmation of vasculitis with extravascular eosinophils
  • Paranasal sinusitis
  • CXR - Pulmonary infiltrates
  • Mononeuritis multiplex
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38
Q

What are the classical symptoms/signs of EGPA?

A
  • Asthma
  • Fever
  • Joint pain/Diffuse muscle pain
  • Weight loss
  • Abdominal/Chest pain
  • Allergic rhinitis/Nasal polyposis
  • Purpura/Nodules/Urticaria/Livedo Reticularis
  • Peripheral neuropathy
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39
Q

A patient presents with the following symptoms, what is the most likely diagnosis? What are the differentials?

A 34-year-old woman with a 2-year history of poorly controlled asthma and allergic rhinitis presents with new right lower extremity weakness. Electromyogram indicates mononeuritis multiplex.

A
  • Churg Strauss - EGPA
  • GPA
  • Polyarteritis Nodosa
  • Rheumatoid Arthritis
  • Infection
  • Malignancy
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40
Q

A patient presents with the following Hx, suggestive of EGPA. What investigations should you carry out?

A 34-year-old woman with a 2-year history of poorly controlled asthma and allergic rhinitis presents with new right lower extremity weakness. Electromyogram indicates mononeuritis multiplex.

A
  • FBC – Eosinophilia
  • CRP/ESR - Increased
  • U&Es – Normal or Increased Cr (Glomerulonephritis)
  • Urine dipstick – Blood ++, Protein ++, RBC casts
  • ANCA – Increased
    • Specifically pANCA + MPO – Increased
  • IgE – Increased
  • CXR – Pulmonary opacities/Infiltrates/Effusions
  • Lung biopsy – Small necrotising granulomas
  • Echo
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41
Q

How is EGPA Managed?

A

Maintenance

  • High dose steroids – Prednisolone
  • IV Ig/Plasmaphresis
  • Rituximab
  • Asthma management - Inhaled Steroids

If Life threatening/Severe:

  • Cyclophosphamide
  • Methotrexate
  • Azathioprine
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42
Q

What is Antiphospholipid syndrome?

A

Systemic autoimmune disease

Leads to hypercoagulability of blood due to antibodies against coagulation factors, and activation of platelets & vascular endothelium

Characterised by:

  • Increased Antiphospholipid antibodies
  • Acquired thrombophilia
  • Increased clotting
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43
Q

What are the antiphospholipid antibodies?

A
  • Lupus Anticoagulant
  • Anticardiolipin Ab
  • Anti-beta 2 glycoprotein 1
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44
Q

What are the key characterisitics of Antiphospholipid syndrome?

A
  • Arterial & venous thrombosis
  • Adverse pregnancy outcomes >3 miscarriages
  • Increased antiphospholipid antibodies
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45
Q

Who classically gets antiphospholipid syndrome?

A
  • Women - Young women of fertile age
  • Black people
  • FHx - Increased risk especially with HLA DR
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46
Q

What are the diagnostic criteria for APS?

A

Present if 1 clinical criteria + 1 laboratory criteria

Clinical criteria:

  • Vascular thrombosis >1 episode of Arterial/Venous/Small vessel thrombosis
  • Pregnancy morbidity – >3 miscarriages at <10wks or at least 1 unexplained death of normal fetus after 10wks

Laboratory criteria:

  • Lupus anticoagulant >2 occasions 12 wks apart
  • Increased Anticardiolipid Ab >2 occasions 12 wks apart
  • Anti B2 Glycoprotein 1 Ab >2 occasions 12 wks apart
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47
Q

What are the symptoms/signs of APS?

A
  • Peripheral artery/vein thrombosis
  • DVT
  • Pregnancy loss at any gestation
  • PE or Pulmonary HTN
  • MI symptoms
  • SLE
  • Livedo reticularis - doesnt disappear on warming
  • Oedema – Due to nephropathy
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48
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differential diagnoses?

A 35-year-old woman has a history of 3 consecutive pregnancy losses before 12 weeks of pregnancy. She had no other known complications during the pregnancies. Further testing reveals a lupus anticoagulant, which is still present on repeat testing 12 weeks later. Physical examination is normal.

A
  • Antiphospholipid syndrome
  • Protein C/S/Antithrombin 3 deficiency
  • Malignancy
  • Contraceptives
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49
Q

A patient presents with the following Hx, suggestive of APS. What investigations should be carried out?

A 35-year-old woman has a history of 3 consecutive pregnancy losses before 12 weeks of pregnancy. She had no other known complications during the pregnancies. Further testing reveals a lupus anticoagulant, which is still present on repeat testing 12 weeks later. Physical examination is normal.

A
  • Lupus Anticoagulant – Increased
  • Anticardiolipin Ab – Increased
  • Anti beta 2 Glycoprotein – Increased
  • FBC – Thrombocytopenia + Haemolytic anaemia
  • ANA – Normal. Increased if SLE is concurrent
  • DsDNA – Normal. Increased if SLE is concurrent
  • U&E – Increased Urea + Creatinine (If neuropathy present)
  • Clotting screen – Negative
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50
Q

How is APS managed?

A

Counselling + Lifestyle management to prevent CVD – Avoid smoking, Healthy diet + exercise

Thrombosis management

  • Acute – Heparin
  • Prophylaxis – Warfarin INR 2-3 or Clopidogrel/Dabigatran

If thrombosis continues:

  • High dose steroids
  • Plasmaphresis
  • Rituximab
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51
Q

What is Rheumatoid Arthritis?

A
  • Chronic Inflammatory Autoimmune condition
  • Inflammation of the synovial joints leading to Joint + Periarticular tissue destruction
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52
Q

Who typically gets Rheumatoid arthritis?

A
  • Women
  • At any age, but peak age is 40’s
  • FHx
  • Smokers
  • Patients with DM
  • HLA DR4
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53
Q

What are the classical symptoms and signs of rheumatoid arthritis?

A
  • Symmetrical polyarthritis - Small joints
  • Heat/Erythema/Swelling of joints
  • Early morning stiffness > 30mins
  • Progressive loss of function
  • Chronic joint pain
  • Fever/Night sweats
  • Weight loss
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54
Q

What are the systemic symptoms associated with rheumatoid arthritis?

A
  • Eyes – Secondary Sjogrens syndrome/Scleritis
  • Skin – Rashes/Nail fold infarcts
  • Neurological – Peripheral nerve entrapment/Neuropathy
  • Respiratory – Pleural/Pulmonary fibrosis
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55
Q

What are the classical signs O/E of Rheumatoid arthritis?

A
  • Bilateral joint inflammation affecting – ICP/MCP/Wrist joints
  • Nail fold infarcts
  • Swan neck deformity
  • Ulnar deviation
  • Boutonnieres/Herbedens nodes
  • Z thumb
56
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 52-year-old woman presents with a 2-month history of bilateral hand and wrist pain, and swelling in her fingers. She has also recently noted similar pain in the balls of her feet. She finds it hard to get going in the morning and feels stiff for hours after waking up. She also complains of increasing fatigue and is unable to turn on and off taps or use a keyboard at work without a significant amount of pain in her hands. She denies any infections before or since her symptoms started.

A
  • Rheumatoid Arthritis
  • Viral arthritis
  • Psoriatic arthritis
  • AS
  • Gout
  • Reactive arthritis
  • SLE/Scleroderma
  • PMR
57
Q

A patient presents with the following Hx suggestive of rheumatoid arthritis. What investigations should be ordered?

A 52-year-old woman presents with a 2-month history of bilateral hand and wrist pain, and swelling in her fingers. She has also recently noted similar pain in the balls of her feet. She finds it hard to get going in the morning and feels stiff for hours after waking up. She also complains of increasing fatigue and is unable to turn on and off taps or use a keyboard at work without a significant amount of pain in her hands. She denies any infections before or since her symptoms started.

A
  • FBC – Anaemia of chronic disease (Normochromic Normocytic) + Reactive thrombocytosis
  • Ferritin – Increased
  • U&E – Normal
  • LFTs – Increased ALP/GGT
  • ESR/CRP – Increased
  • Synovial fluid analysis – Normal
  • Rheumatoid factor – Increased, may be negative
  • Anti-CCP – Increased (More sensitive)
  • ANA – Increased
  • Urine dipstick – Normal
  • X-ray hands & Feet:
    • Soft tissue swelling
    • Periarticular osteopenia
    • Loss of joint space
    • Deformity
58
Q

How is Rheumatoid Arthritis managed?

A
  • Patient education
  • Lifestryle modification – Stop smoking
  • MDT – GP/Rheumatologist/Nurse/Physio

Symptom management

  • Paracetamol
  • NSAIDs – Ibuprofen/Naproxen
  • Gastric protection – Omeprazole
  • Monitor disease – DAS 28 score

Disease modification:

  • Low dose Corticosteroid (Prednisolone/Dexamethasone) + DMARD (Methotrexate/Sulfasalizine)
  • Bone protection – Vitamin D + Calcium supplements

If resistant to DMARDs:

  • Biological therapy – Infliximab/Rituximab
59
Q

What respiratory complications can be caused by Rheumatoid Arthritis?

A
  • Rheumatoid nodules in the lungs – Pleural effusion/pneumothorax/fistula
  • ILD
  • Pulmonary HTN
  • Bronchiolitis/Bronchiectasis
60
Q

You are asked to explain DMARDs to a patient with an inflammatory arthropathy. Specifically Methotrexate. How would you go about this?

A
  • Introduction
  • Check what the patient already knows about their diagnosis
  • Can you get me up to speed on what has happened, what your symptoms were etc?
  • Do you have any allergies?
  • Do you know what this drug does?

[Indication]

  • Rheumatoid arthritis
  • Chemotherapy
  • Severe psoriasis

[How does it work?]

  • Decrease inflammation
  • Suppress the immune system

[Side effects]

  • Mucosal damage - Sore mouth/GI Upset
  • Bone marrow suppression -> Increased risk of infection & neutropenia
  • Alopecia
  • Headache
  • Bruising - due to low platelets
  • Anaemia

[What happens if overdose]

  • Renal impairment
  • Hepatotoxicity
  • Neuro symptoms
    • Headache/Seizure/Coma

[Signpost]

  • If you have signs of infection -> go to A&E due to risk of immunosuppression/Neutropenia

[Avoid]

  • Pregnancy
  • Use contraceptives during use + at least 3 months after stopping

[How to take it]

  • Take ONCE a WEEK - pick which day to take it
  • Must take folic acid when on Methotrexate 5mg
  • This should be taken on a different day
  • For autoimmune conditions take -> Tablet orally
  • Long term treatment to manage rheumatoid arthritis unless patient has SE
  • Can take 4-6 months for the beneficial effects to be seen

[Monitoring]

  • Before starting
    • FBC
    • LFTs
    • U&E
  • Flu & Pneumonia vaccinations
  • Should not be unwell before starting
  • Every 2 weeks until treatment is established
  • 2-3 times a month after this
61
Q

You are asked to explain DMARDs to a patient with an inflammatory arthropathy. Specifically Azathioprine. How would you go about this?

A

Layout:

  • Check what the patient knows
  • Brief history
  • Do they know what the drug is?
  • Indication & Action of the drug
  • Side effects
  • How to take it
  • Monitoring requirements

[Indication & Action of the drug]

Indication:

  • Maintenance of remission of Crohns disease & UC
  • DMARD in Rheumatoid Arthritis + Autoimmune conditions not responding to corticosteroids
  • Prevent organ rejection in transplant patients

Action:

  • Pro drug - it is not pharmacologically active itself, converted to substances that are active
  • Metabolite - 6-Mercaptopurine, further metabolised -> Active substances
  • Inhibit synthesis of purines - Adenine & Guanine
  • Inhibit DNA and RNA replication
  • Note: Lymphocytes are dependent on purine synthesis - affected by azathioprine metabolites
  • Metabolism and elmination requires
  • Xanthine oxidase + TPMT which can be reduced or absent in some people

[Side effects]

  • Bone marrow suppression
  • Leukopenia
  • Increasred risk of infection
  • Nausea
  • Hypersentivity reactions
  • Diarrhoea
  • Vomiting
  • Rash
  • Fever
  • Myalgia
  • Hypotension
  • Pancreatitis
  • Veno-occlusive disease (Rare)
  • Hepatotoxicity
  • Increased risk of tumours such as lymphoma

C/I:

  • Patient with absent TPMT activity
  • Hypersensitivity reactions
  • Pregnancy

Caution:

  • Patients with reduced TPMT activity
  • Hepatic/Renal impairment

[How to take it]

  • Prescribed based on weight, daily in divided doses
  • Dose adjusted to response
  • Treatment may start at high dose - to induce remission
  • Lower dose - maintenance therapy
  • Oral treatment
  • May take time to reach maximal effect

[Monitoring requirements]

Signpost patient to seek help if:

  • Sore throat/fever -> Infection
  • Bruising or bleeding -> Low platelets
  • Rash/Diarrhoea/Vomiting/Abdominal pain -> Hypersensitivity
  • Monitor weekly
    • FBC
  • For first 4 months - then every 3 months
62
Q

You are asked to explain DMARDs to a patient with an inflammatory arthropathy. Specifically Mesalazine/Sulfasalazine. How would you go about this?

A

Layout:

  • Check what the patient knows
  • Brief history
  • Do they know what the drug is?
  • Indication & Action of the drug
  • Side effects
  • How to take it
  • Monitoring requirements

[Indication & Action of the drug]

Indication:

  • 1st line treatment for mild-moderate UC
  • DMARD for Rheumatoid arthritis

Action:

  • Release 5-aminosalicyclic acid (5-ASA) Leads to
  • Anti-inflammatory effects
  • Immunosuppressive effects
  • Exact mechanism of action is not known

[Side effects]

  • GI upset
  • Nausea
  • Dyspepsia
  • Headache
  • Blood abnormalities
  • Leucopenia
  • Thrombocytopenia
  • Renal impairment
  • Oligospermia - reversible decrease in number of sperm
  • Serious hypersensitivity reaction
  • Fever
  • Rash
  • Liver abnormalities

C/I:

  • Not to be used in patients with Aspirin hypersensitivity/Allergy

[How to take it]

  • In UC (Rectal/Rectosigmoid) -> Suppository
  • Once or 12hrly for 4-6 weeks to induce remission
  • In UC (Proximal to rectum) -> Tablet
  • In RA -> Tablet

[Monitoring requirements]

  • Monitor symptoms Ask patient to report
  • Unexplained bleeding
  • Bruising
  • Infective symptoms
  • Monitor
    • Disease activity score & symptoms
    • CRP/ESR - acute phase reactants
    • U&E - renal function
    • FBC
    • LFTs
63
Q

You are asked to explain a drug to a patient with an inflammatory arthropathy. Specifically Systemic corticosteroids (Prednisolone/Dexamethasone). How would you go about this?

A

[Indication & Action of the drug]

Indication:

  • Treat allergic/inflammatory disorders - Anaphylaxis/asthma
  • Suppression of autoimmune disease - IBD/Inflammatory Arthritis
  • Treatment of some cancers - as part of chemotherapy, to reduce tumour associated swelling
  • Hormone replacement in adrenal insufficiency/hypopituitarism

Action:

  • Exert glucocorticoid effects
  • Bind to cytosolic glucocorticoid receptors -> translocate to nucleus -> bind to glucocorticoid response elements (GRE)
  • These GRE -> regulate genre expression
  • Most commonly prescribed to modify the immune response
  • Up-regulate anti-inflammatory genes
  • Down-regulate pro-pro-inflammatory genes (e.g. Cytokines/TNFa)
  • Direct actions on inflammatory cells ->
  • Suppression of circulating monocytes & eosinophils

Metabolic effects

  • Increase gluconeogenesis from increased circulating amnio fatty acids that are released due to catabolism of muscle and fat

Mineralocorticoid effects

  • Stimulate Na+ and water retention
  • K+ excretion in the renal tubule

[Side effects]

  • Immunosuppression - increases risk of severe infection and altered host response
  • Metabolic effects
  • DM
  • Osteoporosis
  • Increased catabolism
  • Proximal muscle weakness
  • Skin thinning
  • Easy bruising
  • Gastritis
  • Mood and behavioural changes
  • Insomnia
  • Confusion
  • Psychosis
  • Suicidal ideation
  • Mineralocorticoid effects
  • HTN
  • Hypokalaemia
  • Oedema
  • Suppression of ACTH secretion Switches off stimulus for normal adrenal cortisol production
    • Adrenal atrophy -> Prevents endogenous cortisol secretion
  • If withdrawn suddenly -> Addisonian crisis
  • Cardiovascular collapse

Long term use:

  • Osteoporosis
  • Bone #
  • Diabetes

Withdrawal

  • Fatigue
  • Weight loss
  • Arthralgia

Caution

  • People with pre-exisitng infection
  • Children - can suppress growth

[How to take it]

  • Withdrawal must be done slowly (Tapered) to avoid Addisonian crisis
  • Can be given Oral/IV/IM
  • May require co-prescription in high risk patients:
    • Bisphosponates
    • PPI
  • Should be taken in morning - mimic natural circadian rhythm of cortisol release, and reduce insomnia
  • Should start to feel better within 1-2 days
  • MUST not stop treatment suddenly
  • Steroid card to be carried at all times

[Monitoring requirements]

  • Monitor efficacy
  • ESR/CRP
  • Adverse effects
  • HbA1c & Glucose
  • DEXA
64
Q

You are asked to explain a drug to a patient with an inflammatory arthropathy. Specifically NSAIDs (Naproxen + Ibuprofen + Etoricoxib). How would you go about this?

A

Layout:

  • Check what the patient knows
  • Brief history
  • Do they know what the drug is?
  • Indication & Action of the drug
  • Side effects
  • How to take it
  • Monitoring requirements

[Indication & Action of the drug]

Indication:

  • PRN for mild-moderate pain
  • Alternative to paracetamol
  • Regular treatment for pain related to inflammation e.g. RA/Severe OA/Acute gout

Action:

  • Inhibit prostaglandin synthesis from arachidonic acid -> Inhibit COX enzyme COX1 + COX2 Cox1 - stimulates prostaglandin synthesis - essential for preservation of the integrity of the gastric mucosa + maintains renal perfusion (dilates afferent glomerular arterioles)
  • Inhibits thrombus formation at vasucular endothelium
  • Cox 2 - Expressed in response to inflammatory stimuli
  • Stimulates production of prostaglandins -> inflammation and pain
  • Main effects of NSAIDs are due to
    • Cox 2 - INHIBITION
    • Cox 1 - INHIBITION

[Side effects]

  • GI toxicity
  • Renal impairment
  • Increased risk of cardiovascular events - MI/Stroke
  • Hypersensitivity reaction
  • Bronchospasm
  • Angiodema
  • Fluid retention - Worsen HTN and Heart failure

Note: Ibuprofen -> Lowest GI risk

  • Naproxen & Ibuprofen -> Lowest risk of cardiovascular events
  • Etoricoxib -> fewer GI SE, increased risk of cardiovascular events

Complications & CI

Avoid in:

  • Severe renal impairment
  • Heart failure
  • Liver failure
  • NSAID hypersensitivity

Caution in:

  • Peptic ulcer disease
  • GI Bleeding
  • CVD
  • Renal impairment

[How to take it]

  • Take orally with food to avoid GI upset
  • Also available as Gel(Topical)/Suppository/Injection
  • Regular treatment for at least 3 weeks required in inflammatory conditions for the effects to be seen
  • In acute pain effects may be seen immediately, stopped when pain is resolved
  • You may be given PPI if you have an increased risk of GI complications:
    • >65yrs
    • Previous PUD
    • CVD/DM
  • Concurrent treatment with Prednisolone/aspirin

[Monitoring requirements]

  • Advise patient to stop treatment and seek medical attention if:
  • GI upset
  • Rash
  • Stop to reduce risk of kidney damage:
    • Acutely unwell
    • Dehydrated
    • Monitor efficacy
    • Pain - scoring system for level of pain
  • Monitor
    • U&Es - if existing renal impairment
65
Q

You are asked to explain a drug to a patient with Gout. Specifically Allopurinol. How would you go about this?

A

Layout:

  • Check what the patient knows
  • Brief history
  • Do they know what the drug is?
  • Indication & Action of the drug
  • Side effects
  • How to take it
  • Monitoring requirements

[Indication & Action of the drug]

Indications

  • Prevent recurrent attacks of gout
  • Prevent uric acid & calcium oxalate renal stones
  • Prevent hyperuricaemia & tumour lysis syndrome associated with chemotherapy

Actions:

  • Xanthase oxidase inhibitor
  • XO normally metabolises xanthine (Produced from purines) -> Uric acid
  • Inhibition of the XO -> Lowers plasma uric acid concentrations
  • Reduces precipitation of uric acid in the joints/kidneys

[Side effects]

  • Generally well tolerated
  • Starting Allopurinol can trigger or worsen an acute attack of gout - through affects on preformed crystals
  • Reduce risk of triggering attack by co-prescribing NSAID or Colchicine in initiation phase
  • Most common SE:Skin rash
  • Mild or can be severe -> SJS or toxic epidermal necrolysis
  • Hypersensitivity syndrome (Life threatening)
  • Fever
  • Eosinophilia
  • Lymphadenopathy
  • Involvement of liver & skin
  • Contraindications Avoid in Acute attack of gout
  • Continue if patient already established
  • Recurrent skin rash
  • Signs of severe hypersensitivity

Caution in:

  • Renal impairment
  • Hepatic impairment

[How to take it]

  • Taken orally - start at low dose and titrate up
  • Co-prescribe NSAID/Colchicine for at least 1 month after normal uric acid levels to avoid triggering acute attack
  • Take after meals
  • Encourage good hydration with approx 2-3L fluid intake daily

[Monitoring requirements]

  • Serum uric acid concentration checked 4 weeks after initiation/change in dose
  • Monitor for rash - stop treatment if rash develops
66
Q

What is Ankylosing Spondylitis?

A
  • Chronic seronegative spondyloarthropathy, involving the axial skeleton
  • Classic: Sacroilitis + Spondylitis
  • Inflammation + Cartilage erosion + Ossification
67
Q

Who typically gets AS?

What are the symptoms/signs?

A
  • European countries
  • Age 20-30
  • Male > Females
  • Associated with psoriasis + IBD
  • HLA B27 linked
  • FHx

Symptoms/Signs:

  • May have subtle symptoms in early disease
  • Insidious onset over several months/years
  • Chronic disease or intermittent flares with periods of remission
  • Morning stiffness >30mins
  • Stiffness + Pain wakes pt up in morning
  • Tenderness over sacroiliac joints
  • Limited range of spinal motion - stiffness
  • Decreased lumbar lordosis – Bamboo spine (Due to fusion)
  • Severe back pain
  • Gluteal muscle atrophy
  • May have peripheral enthesitis:
    • Achilles tendonitis ± Plantar fasciitis
  • Peripheral arthritis - Symmetrical
  • Systemic features:
    • Fever/Fatigue/Weight loss/Sleep disturbance
68
Q

What extra-articular features might a patient with AS experience?

A
  • Anterior uveitis
  • Aortitis of ascending aorta - Aortic regurgitation
  • AV/Complete heart block
  • Restrictive lung disease
  • Renal amyloidosis
69
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 20-year-old man presents to his primary care physician with low back pain and stiffness that has persisted for more than 3 months. There is no history of obvious injury but he is a very avid sportsman. His back symptoms are worse when he awakes in the morning, and the stiffness lasts more than 1 hour. His back symptoms improve with exercise. He has a desk job and finds that sitting for long periods of time exacerbates his symptoms. He has to get up regularly and move around. His back symptoms also wake him in the second half of the night, after which he can find it difficult to get comfortable. He normally takes an anti-inflammatory drug during the day, and finds his stiffness is worse when he misses a dose. He has had 2 bouts of iritis in the past.

A
  • Ankylosing spondylitis
  • Mechanical back pain
  • OA
  • RA/Reactive arthritis
  • Infection – TB
  • Malignancy
70
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 17-year-old boy presents with an 18-month history of pain in his right ankle and both heels, with early morning stiffness and fatigue. He was forced to give up sport, and walking short distances is proving difficult due to heel pain. Examination reveals marked tenderness and swelling over bilateral Achilles tendons.

A
  • Ankylosing spondylitis
  • Mechanical back pain
  • OA
  • RA/Reactive arthritis
  • Infection – TB
  • Malignancy
71
Q

A patient presents with the following Hx suggestive of ankylosing spondylitis, what investigations will you do? How will you manage it?

A 17-year-old boy presents with an 18-month history of pain in his right ankle and both heels, with early morning stiffness and fatigue. He was forced to give up sport, and walking short distances is proving difficult due to heel pain. Examination reveals marked tenderness and swelling over bilateral Achilles tendons.

A

Investigations:

  • Clinical + Radiological diagnosis
  • Low back pain >3 months
  • Improved by exercise, Not relieved by rest
  • Limitation of lumbar spine motion/chest expansion

Pelvis X-ray:

  • Sacro-ilitis at sacroilliac joint
  • MRI – Detect enthesitis
  • HLA B27 – May be positive

Management:

  • x-ray C + T + L spine to assess baseline disease progression
  • No cure
  • Physiotherapy/Exercise programme
  • Hydrotherapy
  • NSAIDs ± PPI (Omeprazole)
  • If not tolerated – Paracetamol ± Codeine
  • Local corticosteroid injections for arthritis/enthesitis/sacroillitis
  • Specialist – TNF alpha inhibitors if severe
72
Q

What is Gout?

A
  • Arthritis due to the deposition of Monosodium Urate (MSU) crystals within joints

Causing:

  • Acute inflammation + Tissue damage
  • Due to either Impaired renal excretion of urate - Under-excreter/Over producer
  • Urate = Metabolite of purines (DNA AA)
73
Q

Who typically gets Gout?

What are the classical symptoms/signs?

A
  • Men > Women
  • Age 30-60yrs
  • Asians
  • Consumption of excess seafood/red meat/alcohol (Beer)
  • Diuretic use
  • Only postmenopausal women

Symptoms/Signs:

  • Acute joint pain (Severe)
  • Fever + Malaise
  • Joint stiffness
  • FHx of gout
  • Swollen + Tender + Erythematous joint
  • Podagra – 1st MTP joint affected, but can also affect other areas
  • Gouty tophi
  • May have joint damage – Restricted ROM + Crepitus + Deformity
74
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 54-year-old man complains of severe pain and swelling in his right first toe that developed overnight. He is limping because of the pain and states that this is the most severe pain he has ever had (‘even covering my foot with the bed sheet hurts’). He has had no previous episodes. His only medication is hydrochlorothiazide for hypertension. He drinks 2 to 3 beers a day. On examination, he is obese. There is swelling, erythema, warmth, and tenderness of the right first toe. There is also tenderness and warmth with mild swelling over the mid foot.

A
  • Gout
  • Sepsis
  • Rheumatoid Arthritis
  • Pseudogout
  • OA
75
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

An 85-year-old man presents with several days of swelling and severe pain in both hands limiting his ability to use his walking frame. He has a history of gout but has not experienced these symptoms before. On examination, he has a temperature of 37.8°C (100.1°F). There is diffuse warmth, mild erythema, and pitting oedema over the dorsum of both hands. There is tenderness and limited hand grip bilaterally. There are multiple nodules around several of the proximal interphalangeal and distal interphalangeal joints, and effusion and tenderness in his left olecranon bursa with palpable nodules.

A
  • Gout
  • Sepsis
  • Rheumatoid Arthritis
  • Pseudogout
  • OA
76
Q

A patient presents with the following Hx suggestive of Gout, what investigations and management should be started?

An 85-year-old man presents with several days of swelling and severe pain in both hands limiting his ability to use his walking frame. He has a history of gout but has not experienced these symptoms before. On examination, he has a temperature of 37.8°C (100.1°F). There is diffuse warmth, mild erythema, and pitting oedema over the dorsum of both hands. There is tenderness and limited hand grip bilaterally. There are multiple nodules around several of the proximal interphalangeal and distal interphalangeal joints, and effusion and tenderness in his left olecranon bursa with palpable nodules.

A

Investigations:

Clinical diagnosis if:

  • Podagra
  • Hyperuricaemia (Serum)

Synovial fluid aspiration - confirm diagnosis:

  • MSU crystals present
  • WCC normal
  • Gram stain + Culture – Negative
  • Serum Uric acid – Increased + 2 weeks after attack

x-ray:

  • Punched out lesions
  • Sclerosis
  • Tophi (Joint destruction)

Management:

Rest + ICE + Elevation

Acute attack:

  • NSAID – Diclofenac/Naproxen
  • Colchicine - 500 micrograms 2-4x daily
  • Lifestyle modifications – Weight loss/Exercise/Diet modification/Decrease alcohol consumption/Increased fluid intake
  • Analgesics – Paracetamol/Co-Codamol

Prophylactic - After >2 attacks a year:

  • Lifestyle modifications
  • Allopurinol - 2 weeks after resolution of attack
  • Colchicine + NSAID (Prevent attack whilst starting + 3 mo)
  • Prednisolone low dose
  • Alternative – Febuxostat
77
Q

What is Pseudogout?

A
  • Inflammation of the joints caused by deposition of Calcium Pyrophosphate (CPP) crystals into the articular + periarticular tissue
  • Acute – Acute inflammatory arthritis of 1 or more joints
  • Chronic – Mimics OA/RA and associated with ongoing inflammation

Leads to:

  • Deposition of crystals in articular hyaline + cartilage fibers
  • CPP produced by chondrocytes, which can break off and cause inflammation + degeneration of cartilage
78
Q

Who classically gets pseudogout?

What are the symptoms/Signs?

A
  • Common in the elderly
  • Can be precipitated by dehydration/hyper-parathyroidism/intercurrent illness/steroid use/hypothyroidism

Symptoms/signs:

  • Asymptomatic – Incidental radiographic finding
  • Acute monoarticular arthritis or Oligoarticular
  • Usually affecting knees/wrists/shoulders/hands
  • Swelling/Erythema
  • Tenderness
  • Increased temperature
  • Pain on movement
  • Fever
  • Similar to acute gout
79
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 72-year-old woman presents with polyarticular joint pain. She has long-standing mild joint pain, but over the last 10 years notes increasing discomfort in her wrists, shoulders, knees, and ankles. She has had several recent episodes of severe pain in 1 or 2 joints, with swelling and warmth of the affected areas. These episodes often last 3 to 4 weeks. Her examination shows severe bony changes consistent with osteoarthritis in many joints, and slight swelling, warmth, and tenderness without erythema in the second and third metacarpophalangeal joints, left shoulder, and the right wrist.

A
  • Pseudogout
  • Acute gout
  • OA
  • Septic arthritis
  • RA
80
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

An 80-year-old man presents with a swollen red wrist, fever, and chills. He recalls falling out of his wheelchair several days ago but seemed well until 24 hours before admission, when he developed pain in his right wrist. His daughter noted fever and some confusion and brought him to the hospital. On examination, he appears ill and has fever to 39ºC (102ºF). There is swelling, tenderness, and redness around the right wrist with oedema over the dorsum of the hand.

A
  • Pseudogout
  • Acute gout
  • OA
  • Septic arthritis
  • RA
81
Q

A patient presents with the following Hx suggestive of pseudogout, what investigations should be ordered? How should it be managed?

An 80-year-old man presents with a swollen red wrist, fever, and chills. He recalls falling out of his wheelchair several days ago but seemed well until 24 hours before admission, when he developed pain in his right wrist. His daughter noted fever and some confusion and brought him to the hospital. On examination, he appears ill and has fever to 39ºC (102ºF). There is swelling, tenderness, and redness around the right wrist with oedema over the dorsum of the hand.

A

Investigations:

  • Serum urate – Normal

Synovial fluid aspirate:

  • Increased WCC
  • Normal glucose
  • Positive CPP crystals

x-ray of affected joint:

  • Linear opacification of articular cartilage
  • Serum Ca+ – Normal
  • Serum PTH – normal

Management:

  • Only if symptomatic:
  • Ice pack + Rest + Elevation
  • Aspiration of joint
  • NSAIDs
  • Intra-articular steroid injections - Dexamethasone
  • Paracetamol
82
Q

What is Reactive arthritis/Reiters syndrome?

A
  • Seronegative spondyloarthropathy/arthritis
  • Associated with inflammatory back pain
  • Additive - migratory arthopathy (Oligoarthritis) + Extra articular features
  • Follows GI/GU infection up to 6 wks prior
83
Q

What is the Reiters triad?

A
  1. Oligoarthritis - large joints arthritis
  2. Urogenital tract infection – Urethritis/Cervicitis
  3. Uveitis/Conjunctivitis
84
Q

What typically causes Reactive arthritis?

A
  • Associated with HLA B27
  • Post enteric infection – Campylobacter/Salmonella/Shigella
  • Post venereal – Chlamydia/HIV Infection
  • TB disease – Poncets disease
85
Q

Who typically gets reactive arthritis?

What are the symptoms/signs?

A
  • Young adults
  • Male > Female
  • HLA B27
  • Commonly caused by GU infections

Symptoms/Signs:

  • <6wks after a GI or GU infection, but may not have infection
  • Acute onset
  • Asymmetrical oligoarthritis - Painful + Swollen + Stiff joints
  • Malaise/Fatigue/Fever
  • Skin change - Erythema nodosum/Balanitis
  • Dactylitis (Dystrophic nail change)
  • Mouth ulcers
  • Abdominal pain + Diarrhoea
  • May have low back back ± Enthesitis
86
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 21-year-old male student presents with a 4-week history of a painful, hot, and swollen left knee, low back pain with bilateral buttock pain, and left heel pain. He denies trauma and states the symptoms began acutely. He denies any fever or any other significant arthralgias. Further review of symptoms indicates the patient was treated for a chlamydia infection after he developed dysuria approximately 8 weeks ago (he was treated with a single dose of azithromycin). He admits to unprotected sexual intercourse with a new partner 2 days before the onset of his dysuria. In addition to pain and swelling, the patient reports that he has developed morning stiffness in the left knee and low back that last more than 1 hour. He continues to have episodes of dysuria. The findings of physical examination are significant for a large effusion of the left knee with warmth. Range of motion is slightly diminished. There is tenderness to palpation of the left heel at the site of the Achilles’ insertion. Laboratory findings are significant for an ESR of 35 mm/hour, and both FBC and uric acid level are within normal limits. He is HLA-B27 positive, rheumatoid factor negative, and ANA negative. The synovial fluid analysis is negative for crystals, with a total nucleated cell count of 22,000 cells/microlitre (65% neutrophils). A urethral swab was positive by PCR for Chlamydia trachomatis 9 weeks ago at initial presentation of dysuria. There was no evidence of gram-negative diplococci on Gram stain.

A
  • Reactive arthritis
  • Ankylosing spondylitis
  • IBD
  • Rheumatic fever
  • Gout
  • Psoriatic arthritis
  • RA
  • Septic Arthritis
87
Q

A patient presents with the following Hx, what investigations would you order? How would you manage it?

A 21-year-old male student presents with a 4-week history of a painful, hot, and swollen left knee, low back pain with bilateral buttock pain, and left heel pain. He denies trauma and states the symptoms began acutely. He denies any fever or any other significant arthralgias. Further review of symptoms indicates the patient was treated for a chlamydia infection after he developed dysuria approximately 8 weeks ago (he was treated with a single dose of azithromycin). He admits to unprotected sexual intercourse with a new partner 2 days before the onset of his dysuria. In addition to pain and swelling, the patient reports that he has developed morning stiffness in the left knee and low back that last more than 1 hour. He continues to have episodes of dysuria. The findings of physical examination are significant for a large effusion of the left knee with warmth. Range of motion is slightly diminished. There is tenderness to palpation of the left heel at the site of the Achilles’ insertion. Laboratory findings are significant for an ESR of 35 mm/hour, and both FBC and uric acid level are within normal limits. He is HLA-B27 positive, rheumatoid factor negative, and ANA negative. The synovial fluid analysis is negative for crystals, with a total nucleated cell count of 22,000 cells/microlitre (65% neutrophils). A urethral swab was positive by PCR for Chlamydia trachomatis 9 weeks ago at initial presentation of dysuria.

A

Investigations:

  • FBC:
    • Normochromic normocytic anaemia
    • Mild leukocytosis
    • Thrombocytosis
  • ESR + CRP – Very high
  • HLA B-27 – Positive
  • RF – Negative
  • ANA – Negative

Joint aspiration:

  • Increased WCC (Polymorphonuclear leukocytes)
  • Stool/throat/GU swab – Identify causative organism
  • STI Screen – for Chlamydia

x-ray:

  • Normal in early stages of disease

Long term disease:

  • Slow periosteal reaction
  • Proliferation at tendon insertion
  • Sacroillitis

Management:

  • Rest affected joints
  • Aspirate synovial effusion
  • Physiotherapy
  • NSAIDs
  • Corticosteroids - Intra-articular/systemic
  • ABx for causative organism
  • Sulfasalazine
88
Q

What is the prognosis for Reactive arthritis?

A
  • Self limiting with resolution within 3-12 months but may persist
  • May develop – Long term destructive arthritis/enthesitis/spondylitis
  • High incidence of recurrence
89
Q

What is Psoriatic arthritis?

A
  • Seronegative arthropathy + Spondyloarthropathy – Associated with HLA-B27
  • Inflammatory arthritis affecting the joints & connective tissue (Autoimmune)
  • Associated with psoriasis of the skin/nails
  • Can occur in absence of skin disease
90
Q

Who classically gets psoriatic arthritis?

What are they symptoms/signs?

A
  • Affects <30% of patients with psoriasis
  • Common in caucasians
  • Age 35-55yrs
  • Men > Women
  • Psoriasis skin changes before arthritis or after

Symptoms/Signs:

  • Arthritis usually within 10yrs of psoriasis diagnosis
  • Joint stiffness >30mins in mornings
  • Improves with use, worse on resting
  • Symmetric/Asymmetric arthritis – Pain + Swelling
  • Relapsing + Remitting
  • May have psoriatic plaques - check navel/behind ears/scalp
  • Joint tenderness
  • Nail changes – Pitting/Onycholysis
  • Dactylitis – Swelling of whole finger
  • May have enthesitis/back involvement
91
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 25-year-old man presents with painful forefeet, a swollen right knee, and a swollen index finger. These symptoms developed over 2 months. He has had a history of psoriasis since the age of 18 years. Examination reveals psoriatic plaques at the knees and elbows, as well as at the posterior hair line and psoriatic nail changes of both fingers and toes. There is a dactylitis of the right index finger and several toes, as well as synovitis of the right knee.

A
  • Psoriatic arthritis
  • RA
  • AS
  • Gout
  • Reactive arthritis
  • Enteropathic arthropathy
  • Septic arthritis
92
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 57-year-old woman presents complaining of pain and stiffness in both knees and the left foot. Past medical history is notable for severe dandruff and an episode of apparent gout in the left first toe 6 months ago, which never resolved. Examination confirms scalp psoriasis, synovitis of the knees, and a dactylitis of the left first toe.

A
  • Psoriatic arthritis
  • RA
  • AS
  • Gout
  • Reactive arthritis
  • Enteropathic arthropathy
  • Septic arthritis
93
Q

A patient presents with the following Hx, suggestive of Psoriatic arthritis. What investigations would you order? How would you manage it?

A 57-year-old woman presents complaining of pain and stiffness in both knees and the left foot. Past medical history is notable for severe dandruff and an episode of apparent gout in the left first toe 6 months ago, which never resolved. Examination confirms scalp psoriasis, synovitis of the knees, and a dactylitis of the left first toe.

A

Investigations:

  • Clinical diagnosis
  • FBC – Normochromic Normocytic anaemia
  • ESR + CRP – Raised
  • RF – Negative, may be false positive
  • Anti-CCP – Negative
  • ANA – Negative

Synovial fluid aspiration:

  • Increased WCC
  • No crystals
  • Serum IgA – Increased, or may be normal

x-ray: Signs of inflammatory arthritis – Bony erosions

MRI – If enthesitis/Sacro-illiac involvement

Management:

  • Patient education
  • Physiotherapy
  • NSAIDs for pain
  • Local steroid injections
  • Methotrexate/Sulfasalazine/Leflunomide
  • Consider TNF inhibitors
94
Q

What is Systemic Lupus Erythematosus?

A
  • Inflammatory multisystem autoimmune condition
  • Affinity of IgG antibodies to double stranded DNA & Nuclear proteins
  • T cell dysregulation of B cells
95
Q

Who classically gets SLE?

What are the symptoms/Signs?

A
  • Females > Males
  • 50-70yrs
  • FHx of SLE
  • Common in asian + black origin
  • HLA DRB1 common

Symptoms/Signs:

  • Relapsing remitting disease
  • Malar rash - sparing nasolabial folds, photosensitive
  • Splenomegaly
  • Lymphadenopathy
  • Weight loss
  • Oral ulcers
  • Fever/Fatigue (Severe + Debilitating)
  • Arthralgia + Early morning stiffness >30mins
  • Headache
  • Raynauds phenomenon
  • Hair loss + Myalgia
96
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 38-year-old white woman presents to the accident and emergency department with 24 hours of dyspnoea and pleuritic chest pain. On further questioning, she reports a 3-year history of Raynaud’s disease, polyarthralgia, and intermittent migraine. Physical examination reveals hypoxia, tachycardia, and normal blood pressure. Ventilation perfusion scanning confirms a pulmonary thromboembolism.

A
  • SLE
  • RA
  • Systemic sclerosis
  • APS
  • HIV
97
Q

A patient presents with the following Hx, what is the most likely diagnosis? What are the differentials?

A 16-year-old black female presents to her general practitioner with symptoms of fatigue, musculoskeletal pain, and a facial rash. On examination she is noted to be thin with malar skin changes. No other abnormality is found.

A
  • SLE
  • RA
  • Systemic sclerosis
  • APS
  • HIV
98
Q

What are the systemic symptoms of SLE?

A

Lungs:

  • Pleurisy/Lung fibrosis

Heart:

  • Pericarditis/HTN/Endocarditis

Renal

  • Asymptomatic nephritis/Peripheral oedema

Neuropsychiatric

  • Anxiety/Depression/Psychosis
99
Q

A patient presents with the following Hx, suggestive of SLE. What investigations would you carry out? How would you manage it?

A 16-year-old black female presents to her general practitioner with symptoms of fatigue, musculoskeletal pain, and a facial rash. On examination she is noted to be thin with malar skin changes. No other abnormality is found.

A

Investigations:

  • ACR criteria if >4 criteria over number of years:
    • Malar rash
    • Photosensitivity
    • Discoid lupus
    • Oral/Nasopharyngeal ulcers
    • Pleuritis/Pericarditis
    • Non erosive arthritis >2 peripheral joints
    • Renal involvement – Increased protein/casts
    • Seizures/Psychosis
    • Positive Antinuclear Abs
  • FBC – Normocytic Normochromic anaemia, Decreased WCC + Platelets
  • ANA – Increased
  • Anti Ds DNA – Positive
  • Anti Sm RNA – Positive
  • U&Es – Increased urea + Creatinine
  • Urinalysis – Haematuria + Casts + Proteinuria
  • CXR – Cardiomegaly/Pleural effusion

Management:

  • Counselling + Patient education
  • Avoid sun
  • Smoking cessation
  • Increase aerobic exercise
  • NSAIDs/Paracetamol for pain relief
  • Corticosteroids - Prednisolone (Only if life threatening)
  • Azathioprine if steroid sparing required
  • Hydroxychloroquine - for skin lesions + arthralgia + myalgia
  • Mycophenolate Mofetil

If Life threatening SLE:

  • High dose prednisolone
  • Cyclophosphamide
  • Alt: Azathioprine + Plasma exchange
100
Q

What is the effect of SLE on fertility/pregnancy?

A

Fertility:

  • Increased oestrogen can exacerbate SLE
  • Prescribe low dose COCP cautiously
  • Increased risk of thrombosis

Pregnancy:

  • Normal fertility + safe pregnancy if mild + stable SLE

Complications:

  • Recurrent miscarriage
  • PET risk
  • IUGR/Pre-term delivery
  • Increased risk of thrombosis
  • Neonatal lupus
101
Q

What is Polymyalgia Rheumatica?

A
  • Inflammatory condition of unknown cause - thought to be a form of GCA
  • May go on to develop GCA
102
Q

Who classically gets PMR?

What are the symptoms/signs?

A
  • Patients >50yrs
  • Women > Men

Symptoms/Signs:

  • Subacute/acute onset of bilateral or severe persistent pain in neck/shoulder or pelvic girdle
  • Symptom duration >2wks
  • Morning stiffness >30mins
  • Bilateral upper arm tenderness, no weakness
  • Low grade fever/weight loss ± anorexia
  • Malaise
  • Depession
  • Oligoarticular arthritis in wrist/MCP/knee
  • Rapid response to steroids
  • Worse on walking/moving
103
Q

A patient presents with the following Hx, what is the most likely diagnosis? what are the differentials?

A 58-year-old woman presents with a 2-week history of fatigue, anorexia, fevers, and bilateral pain and stiffness in the shoulder and hip girdles. These symptoms are worse at night. Upon awakening in the morning, she feels as if she has a bad flu. She reports difficulty getting out of bed in the morning due to stiffness. Her wrists and finger joints are also painful and swollen.

A
  • PMR
  • GCA
  • RA
  • AS
  • SLE
  • OA
  • MSK pain
104
Q

A patient presents with the following symptoms suggestive of PMR, how would you investigate and manage this patient?

A 58-year-old woman presents with a 2-week history of fatigue, anorexia, fevers, and bilateral pain and stiffness in the shoulder and hip girdles. These symptoms are worse at night. Upon awakening in the morning, she feels as if she has a bad flu. She reports difficulty getting out of bed in the morning due to stiffness. Her wrists and finger joints are also painful and swollen.

A

Investigations:

  • If suspecting PMR – Investigate before treatment
  • If suspecting GCA – Start treatment with high dose Prednisolone immediately
  • ESR – increased
  • CRP – Increased
  • FBC – Normal
  • U&E – Normal
  • LFTs – Normal
  • Protein electrophoresis – Normal (Rule out myeloma)
  • TFTs – Normal
  • RF – negative, may be positive
  • Anti ccp – Negative
  • USS of shoulder ± hip

Managment:

  • Steroids – lower dose than GCA
  • Methotrexate + Folic acid – if steroid sparing required
  • Phsyio + OT for assesment
  • Screen for adverse reaction
105
Q

What is GCA?

A
  • Systemic immune mediated granulomatous vasculitis affecting medium + large arteries
  • Particularly the carotid artery + Temporal artery
  • Medical emergency (Risk of vision loss)
106
Q

Who classically gets GCA?

What are the symptoms/Signs?

A
  • Women > Men
  • White people
  • >50yrs old
  • FHx of PMR/GCA

Symptoms/Signs:

  • Recent onset temporal headache
  • Myalgia
  • Malaise/Fever
  • Weight loss ± Anorexia
  • Scalp tenderness/facial pain
  • Transient visual symptoms - Diplopia
  • Jaw/Tongue claudication - gradually during chewing
  • Headache - worse at night
  • Temporal artery palpation - Absent pulse/beaded/tender/enlarged
  • May have vision loss
  • Carotid bruits
  • Fundoscopy – Ischaemia of optic nerve + pallor + oedema of optic disc + cotton wool spots + small haemorrhages
107
Q

A patient presents with the following Hx, what is the most likely diagnosis? what are the differentials?

A 72-year-old white woman presents with partial vision loss in the right eye. She reports bitemporal headache for several weeks, accompanied by pain and stiffness in the neck and shoulders. Review of systems is positive for low-grade fever, fatigue, and weight loss. On physical examination, there is tenderness of the scalp over the temporal areas and thickening of the temporal arteries. Fundoscopic examination reveals pallor of the right optic disc. Bilateral shoulder range of motion is limited and painful. There is no synovitis or tenderness of the peripheral joints. There are no carotid or subclavian bruits, and the blood pressure is normal and equal in both arms. The remainder of the examination is unremarkable.

A
  • GCA
  • Migraine
  • Tension headache
  • PAN
  • Trigeminal neuralgia
  • Takayashu arteritis
  • Malignancy
108
Q

A patient presents with the following symptoms suggestive of GCA, how would you investigate and manage this patient?

A 72-year-old white woman presents with partial vision loss in the right eye. She reports bitemporal headache for several weeks, accompanied by pain and stiffness in the neck and shoulders. Review of systems is positive for low-grade fever, fatigue, and weight loss. On physical examination, there is tenderness of the scalp over the temporal areas and thickening of the temporal arteries. Fundoscopic examination reveals pallor of the right optic disc. Bilateral shoulder range of motion is limited and painful. There is no synovitis or tenderness of the peripheral joints. There are no carotid or subclavian bruits, and the blood pressure is normal and equal in both arms. The remainder of the examination is unremarkable.

A

Investigations:

  • Diagnostic criteria >3 positive:
  • Age of onset >50yrs
  • New onset/type of headache w/localised pain
  • Temporal artery abnormal on palpation
  • ESR >50mm/hr
  • Abnormal temporal artery biopsy
  • Temporal artery biopsy within 2 wks
  • ESR – Raised
  • CRP – May be raised
  • FBC – Normocytic Normochronic anaemia, normal WCC, May have increased platelets
  • ANA – Normal
  • Complement – Normal
  • LFTs – Normal, may have increased ALP
  • Temporal artery USS – May show abnormalities
  • Aortic arch angiography

Management:

  • Immediate - High dose Prednisolone daily
  • if claudication or visual symptoms – Higher dose
  • If visual symptoms – IV Methylprednisolone
  • Refer to opthalmology + rheumatology urgently
  • Low dose aspirin 75mg
    • PPI - Omeprazole
109
Q

What are the complications of GCA?

A
  • Loss of vision – permanent
  • Aneurysms/Dissections/Stenotic lesions of aorta
  • Seizures/CVA
  • Steroid S/E
110
Q

What is Polyarteritis Nodosa?

A
  • Necrotising inflammatory arteritis of medium + small arteries without:
  • Glomerulonephritis
  • Vasculities in arterioles, capillaries or venules
  • No association w/ANCA
  • Can affect any organ but generally spares the pulmonary + glomerular arteries

Caused by:

  • Hep B virus/Unknown cause (Immune mediated)
111
Q

What is Cutaenous polyarteritis nodosa?

A

Less severe form with:

  • Tender S/C nodules
  • Livedo reticularis
  • Cutaneous ulcers
  • Necrosis
  • Usually associated with streptococcal infection
112
Q

Who classically gets PAN?

What are the symptoms/signs?

A
  • All ethnic groups
  • Men > Women
  • Childhood/Adults

Symptoms/Signs:

  • Fever/Weight loss
  • Headache
  • Testicular pain + tenderness
  • Mononeuropathy/Polyneuropathy
  • Elevated U&Es - Urea/Creatinine
  • Livedo reticularis
  • Myalgia/Arthralgia
  • New onset DBP >90mmHg
  • Hep B infection
  • Dilated or constricted blood vessels w/inflammation
  • Granulocyte/mixed leukocyte infiltrate on arterial biopsy
113
Q

A patient presents with the following Hx, what is the most likely diagnosis? what are the differentials?

A 55-year-old man presents with tingling of the left hand and loss of sensation in both lower limbs. He gives a 6-week history of a 5-kg weight loss and fevers. Examination shows mononeuritis multiplex affecting both the common peroneal nerves and the left radial nerve. Investigation reveals a normocytic, normochromic anaemia (haemoglobin 93 d/L [9.3 g/dL]), neutrophilia (WBC count 11.5 x 10^9/L [11,500/microL]), a raised creatinine (2.48 mg/dL), and elevated inflammatory markers (ESR 89 mm/hour, CRP >15.2 nanomol/L [1.6 mg/L]) but normal urinary sediment. Tests are negative for anti-neutrophil cytoplasmic antibodies (ANCA), with no evidence of hepatitis B infection. His blood pressure is 193/103 mmHg. Sural nerve biopsy demonstrates a transmural vascular inflammatory infiltrate with a mixture of macrophages, lymphocytes, and neutrophils plus evidence of focal and segmental necrotising vasculitis with fibrinoid necrosis. Multiple aneurysms are seen on renal angiography.

A
  • PAN
  • Viral/Bacterial/Parasitic infection
  • SLE/RA
  • GCA/HSP/Wegners
  • Pyoderma gangrenosum
  • Crohns disease
114
Q

A patient presents with the following Hx, what is the most likely diagnosis? what are the differentials?

A 44-year-old woman presents with a 3-month history of abdominal pain, fever, and weight loss. The abdominal pain is cramping in nature and occurs 30 minutes after eating. Abdominal examination is unremarkable. She has widespread purpuric lesions on her lower limbs. Blood tests reveal elevated inflammatory markers (ESR 93 mm/hour, CRP >15.2 nanomol/L [1.6 mg/L]) and raised transaminases (ALT 300 units/L). ANCA is negative. HbeAg and HbsAg are positive, with raised hepatitis B DNA. A liver ultrasound examination is normal. A full-thickness skin biopsy of the purpuric lesions is reported as showing segmental necrotising vasculitis of medium vessels with fibrinoid necrosis. A mesenteric angiogram demonstrates multiple aneurysms involving the superior and inferior mesenteric arteries.

A
  • PAN
  • Viral/Bacterial/Parasitic infection
  • SLE/RA
  • GCA/HSP/Wegners
  • Pyoderma gangrenosum
  • Crohns disease
115
Q

A patient presents with the following symptoms suggestive of PAN, how would you investigate and manage this patient?

A 44-year-old woman presents with a 3-month history of abdominal pain, fever, and weight loss. The abdominal pain is cramping in nature and occurs 30 minutes after eating. Abdominal examination is unremarkable. She has widespread purpuric lesions on her lower limbs. Blood tests reveal elevated inflammatory markers (ESR 93 mm/hour, CRP >15.2 nanomol/L [1.6 mg/L]) and raised transaminases (ALT 300 units/L). ANCA is negative. HbeAg and HbsAg are positive, with raised hepatitis B DNA. A liver ultrasound examination is normal. A full-thickness skin biopsy of the purpuric lesions is reported as showing segmental necrotising vasculitis of medium vessels with fibrinoid necrosis. A mesenteric angiogram demonstrates multiple aneurysms involving the superior and inferior mesenteric arteries.

A

Investigations:

  • FBC – Increased WCC (Neutrophils), Normochromic Normocytic anaemia, Increased platelets
  • CRP + ESR – Increased
  • U&E – Increased creatinine if renal involvement
  • Urine dipstic – Increased protein or normal
  • LFTs – Increased enzymes (Due to Hep B or ischaemia)
  • HBV serology – May be Positive or normal
  • ANCA – Negative
  • Skin/Muscle biopsy – Necrotising inflammation
  • Arteriography – microaneurysms in small/medium sized arteries

Management:

  • Corticosteroids
  • Relapse – Cyclophosphamide

If active Hep B

  • Antivirals
  • Plasma exchange
  • Children – IV Ig + Aspirin

If C-PAN:

  • Mild – NSAIDs + Colchicine
  • Severe – Prednisolone
116
Q

What are the complications of PAN?

A
  • HTN induced AKI
  • Mortality
117
Q

What is Kawasaki disease?

A
  • Idiopathic self limiting systemic vasculitis – autoimmune-mediated
  • Affects children between ages of 6months - 5 years most commonly
  • Predominantly affects children of Asian origin - Japanese/Chinese populations
  • Major complication is -> Coronary artery aneurysm formation

Acute -> Subacute -> Convalescent

118
Q

Who classically gets Kawasaki disease?

What are the symptoms/signs?

A
  • More common in East Asian children
  • Peak incidence is between ages of 18-24 months, but most common in children <5yrs
  • Boys > Girls

Symptoms/Signs:

  • Fever lasting >5 days, with abrupt onset
  • Temp >39 deg
  • Marked irritability of the child
  • Erythema, Swelling, and desquamation affecting the skin of the extremities
  • Bilateral conjunctivitis
  • Widespread non-vesicular Rash
  • Inflammation of the lips, mouth, and/or tongue
  • Strawberry tongue (Due to erythema & prominent papillae)
  • Cervical lymphadenopathy >1.5cm in size
  • Unilateral, non-tender, affecting the anterior cervical chain
  • Lethargy
  • Urethritis
  • Diarrhoea & Vomiting/Abdominal pain
  • Myalgia/Arthralgia/Arthritis
119
Q

A patient presents with the following Hx, what is the most likely diagnosis? what are the differentials?

A previously healthy 1-year-old girl was admitted to a children’s hospital with a 7-day history of spiking fever up to 39.5°C (103°F). Three days after the onset of fever she developed left-sided neck swelling and nappy rash, and became progressively fussy and irritable. She was seen in an emergency department, diagnosed with cervical adenitis, and sent home on oral antibiotics. The mother noted continued irritability, high fever, and decreased oral intake. On subsequent admission she was extremely irritable, with a temperature of 38.9°C (102°F), heart rate of 140 beats per minute, respiratory rate of 40 breaths per minute, and blood pressure 110/54 mmHg. There were no signs of nuchal rigidity. Both palpebral and bulbar conjunctivae were deep red and injected, lips were dry and crusted, the oropharynx hyperaemic with some areas of ulcerated mucosa, and the tongue papillae were enlarged and red (strawberry appearance). Examination of the neck revealed a mildly tender left unilateral mass, measuring 4 cm. The skin showed a generalised polymorphous, erythematous, macular, blanching rash, in addition to severely red and desquamated perineal region. Her extremities, especially palms and soles, were swollen, red, and mildly tender.

A
  • Kawasaki disease
  • Measles
  • Rubella
  • Infectious mononucleosis
  • Parvovirus
  • Erythema multiforme
  • Meningitis
  • SJS
120
Q

A patient presents with the following symptoms suggestive of Kawasaki disease, how would you investigate and manage this patient?

A

Investigations:

  • Clinical diagnosis
  • Presence of fever and diagnostic features
  • Urinalysis
  • Sterile pyuria ± proteinuria
  • FBC
  • May show leukocytosis & neutrophilic
  • Anaemia
  • Thrombocytosis
  • ESR/CRP - Elevated
  • LFTs
  • Elevated ALT/AST
  • Elevated bilirubin
  • ECG
  • May show conduction abnormalities due to carditis
  • Echo
  • Dilatation and aneurysm of coronary arteries

Management:

  • Bed rest in hospital - due to risk of myocardial event
  • Aspirin -> reduce fever and myocardial inflammation
  • IV Ig -> prevent cardiac damage
  • Follow up echo - to determine if there are coronary artery complications

If cardiac complications

  • PCI and CABG
121
Q

What is Takayashu’s arteritis?

A
  • rare autoimmune vasculitic disorder which predominantly affects young women
  • chronic granulomatous vasculitis affecting large arteries: primarily the aorta and its main branches
  • can cause stenosis, occlusion, and aneurysm formation
122
Q

Who classically gets Takayashus?

What are the symptoms/signs?

A
  • rare
  • more common in Asia, especially Japan, than it is in Western countries
  • Women > Men
  • most common in the age range 15-20

symptoms/signs:

  • direct consequence of arterial insufficiency due to the inflammation
  • Fever, fatigue, weight loss.
  • Arthralgia and nonspecific pains.
  • Tenderness overlying the affected arteries
  • Vascular - claudication of the jaw or extremities, back pain (due to involvement of the aorta), syncope (rare), hypertension (the most common presentation in children)
  • Neurological - dizziness, headaches, transient ischaemic attacks (TIAs), visual disturbance, seizures, stroke.
  • Cardiac - angina, dyspnoea (from congestive cardiac failure - the primary cause of death).
  • Pulmonary - haemoptysis, pleuritis
  • Gastrointestinal - abdominal pain from ischaemia
  • systolic blood pressure (SBP) difference >10 mm Hg between arms.
  • Peripheral pulses may not be palpable.
  • Arterial bruits over any large artery and bruit of aortic regurgitation.
123
Q

A patient presents with the following Hx, what is the most likely diagnosis? what are the differentials?

A 28-year-old woman presents with new left-arm pain. She was previously well but for 2 months has had episodes of low-grade fever, night sweats, and arthralgia. She works as a shop assistant and has noticed left-arm pain when she stocks shelves. Her only medication is an oral contraceptive. She does not smoke cigarettes. On examination, her blood pressure is 126/72 in her right arm, but it cannot be measured in her left arm. The left radial pulse cannot be detected. There is a bruit over the left subclavian artery. Carotid pulses are normal but there is a bruit over the right carotid artery. Femoral and pedal pulses are normal and no abdominal bruits are heard. The left hand is cool but has no other evidence of ischaemia.

A
  • Takayashu’s arteritis
  • Acute lymphoblastic leukaemia.
  • Hodgkin’s disease.
  • Polyarteritis nodosa.
  • Rheumatic fever.
  • Giant cell arteritis.
  • Buerger’s disease.
  • Systemic lupus erythematosus.
  • Fibromuscular dysplasia
124
Q

A patient presents with the following Hx, what is the most likely diagnosis? what are the differentials?

A 39-year-old woman presents with headaches of insidious onset over 3 months. She has lost 3 kilograms during this time but feels otherwise well. On examination, bilateral blood pressures taken in the arms are 190/110 on the right and 200/110 on the left. She is taking a multivitamin but no other medications. For the past 20 years she has smoked 10 cigarettes a day. Urinalysis reveals estimated protein of 360 mg/24 hour.

A
  • Takayashu’s arteritis
  • Acute lymphoblastic leukaemia.
  • Hodgkin’s disease.
  • Polyarteritis nodosa.
  • Rheumatic fever.
  • Giant cell arteritis.
  • Buerger’s disease.
  • Systemic lupus erythematosus.
  • Fibromuscular dysplasia
125
Q

A patient presents with the following symptoms suggestive of Takayashu’s, how would you investigate and manage this patient?

A 39-year-old woman presents with headaches of insidious onset over 3 months. She has lost 3 kilograms during this time but feels otherwise well. On examination, bilateral blood pressures taken in the arms are 190/110 on the right and 200/110 on the left. She is taking a multivitamin but no other medications. For the past 20 years she has smoked 10 cigarettes a day. Urinalysis reveals estimated protein of 360 mg/24 hour.

A

Investigations:

  • Diagnostic criteria:
    • Angiography (CT/MRI/DSA) of aorta + main branches + pulmonary arteries
    • Aneurysm dilatation
    • Stenosis/Occlusion
    • Major diagnostic:
    • Signs/symptoms > 1month
    • Claudication of limbs
    • Palpitations
    • Absence of pulses or differential pulse between arms >10 mm Hg
    • Cervical pain.
    • Fever.
    • Amaurosis fugax.
    • Blurred vision.
  • FBC – normocytic anaemia
  • ESR – May be increased
  • Albumin – Decreased
  • CRP – Increased
  • CTA – Segmental narrowing/occlusion/dilatation of aorta

Management:

  • Prednisolone
  • Bone protection – Bisphosphonates
  • Immunosuppression – Azathioprine/Methorexate/Cyclophosphamide
  • Biologics – Rituximab
  • Aspirin - reduce ischaemic events
  • HTN management
  • May require surgery
126
Q

What are the complications of Takayashu’s?

A
  • Loss of vision
  • Hypertension
  • Stroke
  • Aortic regurgitation
  • Myocardial infarction
127
Q

What is Systemic Sclerosis/Scleroderma?

A

Multisystem autoimmune disease with:

  • Increased fibroblast activity - abnormal growth of connective tissue
  • Vascular damage
  • Fibrosis in skin/GIT/Hear/Lungs/Other organs

Vascular manifestations:

  • Raynauds
  • Ischaemia of extremities
  • Pulmonary artery HTN
  • Renal disease
128
Q

What are the different types of scleroderma?

A
  • Limited cutaneous systemic sclerosis:
  • Most common type
  • Affects skin on face/forearms/lower legs

CREST:

  • Calcinosis
  • Raynauds
  • Eosophageal dysmotility
  • Sclerodactylyl (Fibrosis of fingers)
  • Telangectasia

Morphoea – Localised scleroderma

Diffuse cutaneous systemic sclerosis:

  • Less common
  • Affects upper arms/thighs/trunk
  • High risk of mortality
129
Q

Who classically gets Scleroderma?

What are the symptoms/signs?

A
  • FHx increases risk
  • Infectious diseases
  • Chemicals
  • present in all ethnic groups
  • Women > Men
  • Peak age >40yrs

Symptoms/Signs:

  • Raynauds phenomenon
  • Skin hardening on skin + hands
  • Fatigue
  • Myalgia/Arthralgia/Stiffness
  • Hand swelling - Dactylyl
  • Weight loss
  • Dilated nail bed capillaries
  • Limited – Slow onset
  • Diffuse – Rapid onset
  • Telangectasia
  • Heartburn/reflux oesophagitis
  • Pulmonary fibrosis/arterial HTN
  • ANCA associaed glomerulonephritis
130
Q

A patient presents with the following Hx, what is the most likely diagnosis? what are the differentials?

A 38-year-old woman presents with Raynaud’s phenomenon for the past 5 years. She also has a history of digital ulcers and GORD. Physical examination reveals telangiectasias on the hands. She has sclerodactyly. Digital pits are present with no active ulcers.

A 35-year-old woman presents complaining of puffy hands and feet for the past 3 months. She noted the onset of Raynaud’s phenomenon 6 months ago. Examination confirms the presence of puffy hands and feet, with subtle skin thickening of the fingers and dorsum of the hands

A
  1. Limited cutenous systemic sclerosis
  2. diffuse cutaenous sclerosis
  • Morphea
  • Dermatomyositis
  • SLE
  • Raynauds
  • Amyloidosis
131
Q

A patient presents with the following symptoms suggestive of Scleroderma, how would you investigate and manage this patient?

A 35-year-old woman presents complaining of puffy hands and feet for the past 3 months. She noted the onset of Raynaud’s phenomenon 6 months ago. Examination confirms the presence of puffy hands and feet, with subtle skin thickening of the fingers and dorsum of the hands

A

Investigations:

  • FBC – Anaemia, may be reactive increased WCC
  • ESR + CRP – Increased
  • LFTs
  • U&Es – Normal or increased creatinine
  • ANA – Positive
  • Anti Scl 70 – Positive
  • Anti centromere – Positive in Limited SSc
  • Anti RNA poylmerase 3 Ab – Positive in Diffuse SSc

Management:

  • No cure
  • Monitor BP
  • Lung + Cardiac+ Renal function
  • Patient education
  • Home exercise + stretching of skin
  • Avoid smoking
  • Emollients for skin dryness + itching
  • Nifedipine for Raynauds
  • OT
  • Methrotrexate/Mycophenilate Mofetil
132
Q

What can be seen in the following image?

A

Dactylitis/Dactylyl

133
Q

What can be seen in the following image?

A

Livedo Reticularis

134
Q

What can be seen in the following image?

A

Malar Rash - as in SLE

135
Q

What can be seen in the following image?

A

Raynauds

Year 4 M&M (7 decks)

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