Rheumatology Flashcards
What are the joint pathologies dealth with in rheumatology?
- Rheumatoid arthritis
- Gout
- Pseudogout
- Ankylosing spondylitis
- Psoriatic arthritis
- Reactive arthritis
- IBD related arthropathy
What are the connective tissue diseases dealth with in rheumatology?
- SLE/APS
- Scleroderma
- Myositis
- Sjogrens syndrome
- Fibromyalgia
What are the small vessel vasculitis dealth with in rheumatology?
- GPA
- EGPA
- Microscopic polyangitis
- Drug induced vasculitis
What are the medium vessel vasculitis dealth with in rheumatology?
- Polyarteritis Nodosa
- Kawasaki disease
- Behcets disease
What are the large vessel vasculitis dealth with in rheumatology?
- GCA
- PMR
- Takayashu’s arteritis
What signs can be seen in this example of a hand?
Rheumatoid arthritis
- Ulnar deviation
What signs can be seen in this example of a hand?
Rheumatoid arthritis
- Bouchards nodes (Proximal)
- Herbedens nodes (Distal)
What signs can be seen in this image?
Psoriatic nail changes
- Nail pitting
- Onycholysis - Broken nails
- Subungal hyperkeratosis
What signs can be seen in this image?
Gout
- Gouty tophi
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?
Rheumatoid arthritis
- soft tissue swelling
- Bone erosion
- Bone displacement
- Loss of joint space
What is Fibromyalgia?
- Chronic pain disorder
- Cause not known - possibly due to genetics & abnormalities in stress response system
- Peripheral and central hyperexcitability + altered pain perception
Who commonly gets Fibromyalgia?
- Women
- Age 20-50yrs
- Linked to failing to complete eduction, low income, divorce
What are the classic symptoms/signs from a person with Fibromyalgia?
- Widespread pain at multiple sites
- Low back pain
- Associated with fatigue, relieved by rest
- Morning stiffness generally
- Headaches
- Anxiety & depression
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.
- Fibromyalgia
- Chronic fatigue syndrome
- Hypothyroidism
- Vitamin D deficiency
- PMR
- Polymyositis
You suspect a patient may have fibromyalgia, what investigations will you carry out?
- 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
How is Fibromyalgia managed?
- No cure - aim is to reduce physical & mental symptoms, increase function
- MDT management
- Exercise programmes
- CBT
- Paracetamol/weak opioids
What is Sjogrens syndrome?
Autoimmune conditon
Lymphocyte infiltration of the exocrine glands leading to:
- Dry eyes (Keratoconjunctivitis sicca)
- Dry mouth (Xerostomia)
- Enlargement of the parotid glands
What are the classical symptoms of Sjogrens syndrome?
- Dry eyes (Keratoconjunctivitis sicca)
- Dry mouth (Xerostomia)
- Enlargement of the parotid glands
How is Sjogrens syndrome classified?
- Primary – Occurs by itself in isolation
- Secondary – Occurs with other autoimmune condition
Who typically gets Sjogrens syndrome?
- Women
- Age 30-40yrs old
- HLA-DR8 positive
What is the classic presentation (Symptoms/signs) of Sjogrens syndrome?
- 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
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.
- Sjogrens syndrome
- Amyloidosis
- SLE
- Rheumatoid arthritis
- Chronic pancreatitis
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.
- 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
A patient is confirmed to have Sjogrens syndrome, how is this treated?
Symptomatic treatment
- Artificial tears
- Artificial saliva
- Vaginal lubricants
- Emollients for dry skin
What is Wegeners Granulomatosis (GPA)?
Granulomatosis w/polyangiitis
Vasculitis due to autoimmune inflammatory process affecting:
- Endothelial cells
- Small/Medium vessels + arterioles
Due to genetic + environmental factors
What does GPA typically affect?
- Ear, Nose, Throat
- Lungs
- Kidneys
- Can also affect - Joints/Sinuses/Eyes/Skin
How does GPA cause the symptoms it does?
- Frank granuloma development ± necrotising inflammation
- Tissues show little/no immune deposits
- In kidney – Pauci-immune glomerulonephritis
- T cells involved + B cell proliferation
Who typically gets GPA?
- Males, but incidence is nearly same in females
- Peak age 30-50yrs
- FHx
- Previous Parvovirus or Staph Aureus infection
What is the classifcal presentation (Symptoms/Signs) of GPA?
- 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
Which body systems are classically affected in GPA?
- Nose - Bloody rhinorrhea
- Skin - Rashes
- Respiratory system - Cough/dyspnoea
- Kidneys - Haematuria
- Eyes - Inflammation
- Nerves - Mononeuritis multiplex
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.
- GPA/Wegeners Granulomatosis
- Anti-GBM antibody disease
- Mixed connective tissue disease
- SLE
- Churg-Strauss syndrome
- PAN
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.
- 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)
How is a patient with GPA managed?
- 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
What complications can arise from GPA?
- AKI
- Respiratory failure
- Chronic conjunctivitis
- Nasal septum perforation
What is Churg-Strauss syndrome?
- Eosinophilic granulomatosis with Polyangiitis (GPA)
- Diffuse autoimmune vasculitis affecting small/medium sized arteries
- Associated with asthma
Which body systems are affected by EGPA?
- Coronary vessels
- Cerebral vessels
- Respiratory system - Asthma
- GIT – Bowel ischaemia/Perforation
- Kidneys – HTN/Glomerulonephritis/CKD
What are the Diagnostic criteria for EGPA?
>4 symptoms – EGPA diagnosis
- Asthma (Wheezing/Expiratory Rhonchi)
- Eosinophilia >10%
- Histological confirmation of vasculitis with extravascular eosinophils
- Paranasal sinusitis
- CXR - Pulmonary infiltrates
- Mononeuritis multiplex
What are the classical symptoms/signs of EGPA?
- Asthma
- Fever
- Joint pain/Diffuse muscle pain
- Weight loss
- Abdominal/Chest pain
- Allergic rhinitis/Nasal polyposis
- Purpura/Nodules/Urticaria/Livedo Reticularis
- Peripheral neuropathy
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.
- Churg Strauss - EGPA
- GPA
- Polyarteritis Nodosa
- Rheumatoid Arthritis
- Infection
- Malignancy
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.
- 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
How is EGPA Managed?
Maintenance
- High dose steroids – Prednisolone
- IV Ig/Plasmaphresis
- Rituximab
- Asthma management - Inhaled Steroids
If Life threatening/Severe:
- Cyclophosphamide
- Methotrexate
- Azathioprine
What is Antiphospholipid syndrome?
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
What are the antiphospholipid antibodies?
- Lupus Anticoagulant
- Anticardiolipin Ab
- Anti-beta 2 glycoprotein 1
What are the key characterisitics of Antiphospholipid syndrome?
- Arterial & venous thrombosis
- Adverse pregnancy outcomes >3 miscarriages
- Increased antiphospholipid antibodies
Who classically gets antiphospholipid syndrome?
- Women - Young women of fertile age
- Black people
- FHx - Increased risk especially with HLA DR
What are the diagnostic criteria for APS?
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
What are the symptoms/signs of APS?
- 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
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.
- Antiphospholipid syndrome
- Protein C/S/Antithrombin 3 deficiency
- Malignancy
- Contraceptives
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.
- 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
How is APS managed?
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
What is Rheumatoid Arthritis?
- Chronic Inflammatory Autoimmune condition
- Inflammation of the synovial joints leading to Joint + Periarticular tissue destruction
Who typically gets Rheumatoid arthritis?
- Women
- At any age, but peak age is 40’s
- FHx
- Smokers
- Patients with DM
- HLA DR4
What are the classical symptoms and signs of rheumatoid arthritis?
- 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
What are the systemic symptoms associated with rheumatoid arthritis?
- Eyes – Secondary Sjogrens syndrome/Scleritis
- Skin – Rashes/Nail fold infarcts
- Neurological – Peripheral nerve entrapment/Neuropathy
- Respiratory – Pleural/Pulmonary fibrosis