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Finals > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Describe rheumatoid arthritis

A

Symmetrical synovial inflammation (synovitis) causing a polyarthritis

Chronic inflammation of the synovial lining of the joints, tendon sheaths and bursa

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2
Q

Name two gene associations of RA

A

HLADR4 - often present

HLADR1 - occasionally present

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3
Q

Describe rheumatoid factor

A

Autoantibody present in 70% of RA patients

Targets the Fc portion of the IgG antibody

Causes activation of the immune system against the patients own IgG causing systemic inflammation

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4
Q

Describe cyclic citrullinated peptide antibodies

A

Autoantibodies that are more sensitive and specific to rheumatoid arthritis than rheumatoid factor
Often predate the development of RA

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5
Q

Describe the presentation of Rheumatoid arthritis

A

Symmetrical distal polyarthropathy
Joint pain, swelling and stiffness of the small joints of hands and feet, wrist, ankle, MCP and PIP, knees, shoulders and elbows

Systemic symptoms - fatigue, weight loss, flu like illness, muscle aches and weakness

Pain is worse after rest

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6
Q

Describe palindromic Rheumatism

A

Self limtiing short episodes of inflammatory arthritis with joint pain, stiffness and swelling typically only affecting a few joints
Lasts 1-2 days and then completely resolves
Having positive antibodies indicates it may progress into RA

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7
Q

Which condition involves painful and swollen DIP joints

A

Osteoarthritis

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8
Q

Name some signs you may see in the hands of someone with RA

A

Z shape deformity of the thumb - IP and CMC flexion with MP hyperextension

Swan neck - Hyperextension of the PIP joint and flexion of the DIP

Boutonnieres deformity - hyperextension of the DIP joints and flexion of the PIP

Ulnar deviation of the fingers at the MCP joints

Palpation of the synovium in around joints when disease is active will give a body feeling related to the inflammation and swelling

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9
Q

List some extra-articular manifestations of RA

A 
Pulmonary fibrosis
Bronchiolitis obliterans
Feltys syndrome 
Secondary Sjogren's - sicca syndrome 
Anaemia of chronic disease
Cardiovascular diseaase
Episcleritis and scleritis
Rheumatoid nodules 
Lymphadenopathy 
Carpal tunnel syndrome 
Amyloidosis
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10
Q

List the investigations for rheumatoid arthritis

A

Rheumatoid factor
If RF negative, check anti-CCP antibodies
Inflammatory markers such as CRP and ESR
X-ray of hands and feet
Ultrasound scan of the joints can be used to evaluate and confirm synovitis

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11
Q

Describe the Xray changes in RA

A

Joint destruction and swelling
Soft tissue swelling
Bony erosions

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12
Q

What is the American college of rheumatology diagnosis of RA based on

A

The joints that are involved - more and smaller score higher
Serology - RF and anti-CCP
Inflammatory markers - CRP and ESR
Duration of symptoms - more/less than 6 weeks

Scores are added and a score greater than 6 indicates a diagnosis of Rheumatoid arthritis

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13
Q

Describe a DAS28 score

A

Assessment of 28 joints
Points are given for swollen joints, tender joints and ESR/CRP result
Useful in monitoring disease activity and response to treatment

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14
Q

Describe features which indicate a worse prognosis for RA

A 
Younger onset
Make
More joints and organs affected
Antibodies
Erosions
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15
Q

Describe the management of Rheumatoid arthritis

A

MDT
Short course of steroids at initial presentation and during flares
NSAIDs/COX2i (coxibs) but risk GI bleeding so either avoided or prescribed with PPI

Monotherapy with methotrexate, leflunomide or sulfalazine, hydroxychloroquine may be considered in mild disease

2nd line - any 2 of the above combination of DMARDs

3rd line - methotrexate and a biological therapy (TNFi)

4th line - methotrexate plus rituximab

Surgery in very severe joint deformity

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16
Q

Which rheumatoid drugs are safe in pregnancy

A

Sulfalazine and hydroxychloroquine

Pregnancy improves RA symptoms - natural production of steroid hormones

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17
Q

Describe how rituximab works

A

Antibody which binds to the CD20 portion of B cells

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18
Q

How does methotrexate work

A

Interferes with folate metabolism and suppresses certain components of the immune system

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19
Q

What is prescribed with methotrexate

A

Folic acid 5mg taken once a week on a different day to the methotrexate

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20
Q

List some side effects of methotrexate

A

Mouth ulcers and mucositis
Bone marrow suppression
Liver toxicity
Teratogenic - also avoid before conception

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21
Q

Describe the mechanism of action of leflunomide

A

Immunosuppressant by interfering with pyrimidine which is needed for RNA/DNA

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22
Q

List some side effects of leflunomide

A 
Mouth ulcers/mucositis
Hypertension
Rash
Peripheral neuropathy
Teratogenic
Bone marrow suppression
Liver toxicity
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23
Q

Give the side effects of sulfalazine

A

Temporary male infertility

Bone marrow suppression

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24
Q

How does hydroxychloroquine work

A

Immunosuppressant which interferes Toll like receptors, disrupting antigen presentation and increasing the pH of the lysosomes of the immune cells

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25
Q

List some notable side effects of hydroxychloroquine

A

Nightmares
Reduced visual acuity
Liver toxicity
Skin pigmentation

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26
Q

How do anti-TNF drugs work

A

Block TNF which normally stimulates inflammation

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27
Q

List some side effects of anti-TNF drugs

A

Vulnerability to severe infection and sepsis

Reactivation of TB and hep B

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28
Q

List some side effects of rituximab

A 
Vulnerability to severe infection and sepsis
Night sweats
Thrombocytopenia
Peripheral neuropathy
Liver and lung toxicity
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29
Q

What does joint aspiration in rheumatoid arthritis show

A

High WBC count - polymorphonuclear neutrophils
Yellow, cloudy
Absence of crystals

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30
Q

Describe psoriatic arthritis

A

Inflammatory arthritis associated with psoriasis

Seronegative spondyloarthropathy

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31
Q

Describe a symmetrical polyarthritis pattern of psoriatic arthritis

A

Presents similarly to RA and more common in women

Hand, wrists, ankles and DIP joints are affected

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32
Q

Describe an asymmetrical pauciarthrits pattern of psoriatic arthritis

A

Mainly the digits and feet

Few joints

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33
Q

Describe the spondylitic pattern of psoriatic arthritis

A 
Back stiffness
Sacroiliitis 
Atlanto-axial joint involvement 
Spine
Achilles tendon 
Plantar fascia
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34
Q

Describe some signs of psoriatic arthritis

A

Plaques of psoriasis on skin
Pitting of the nails
Onycholysis - separation of the nail from the nail bed
Dactylitis - inflammation of the full finger
Enthesitis - inflammation where points of tendons insert onto bone
Eye disease - conjunctivitis and anterior uveitis
Aortitis
Amyloidosis

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35
Q

Describe a PEST (psorisasis epidemiogical screening tool)

A 
Several questions about joint pain
Swelling
History of arthritis
Nail pitting 
High score triggers referral to rheumatologist
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36
Q

List the X-ray changes of psoriasis

A

Periostitis - inflammation of the periosteum - irregular outline of bone

Anklyosis - bones join together causing stiffening

Osteolysis - destruction of bone

Dactylitis - inflammation of the whole digit which shows as soft tissue swelling

Pencil in cup - central erosion of the bone besides the joints

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37
Q

Describe arthritis mutilans

A

Most severe form of psoriatic arthritis
Occurs in the phalanxes
Osteolysis of the bones around the joints in the digits
Leads to progressive shortening of the digit
Skin then folds as the digit shortens giving appearance of telescopic finger

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38
Q

Describe the management of psoriatic arthritis

A

NSAIDs for pain

DMARDs - methotrexate, leflunomide or sulfasalazine

Anti-TNF medications

Ustekinumab - IL 12 and 23 monoclonal antibody

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39
Q

Describe reactive arthritis

A

Synovitis occurring in joints as a reaction to a recent infective trigger
Acute monoarthritis
Seronegative spondyloarthropathy - HLAB27 link

Bilateral conjunctivitis, anterior uveitis, urethritis, keratoderma blenorrhagica (waxy yellow/brown papules on palms and soles) and balanitis may occur

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40
Q

What are the two most common infections that trigger reactive arthritis

A

Gastroenteritis

STI - chlamydia or gonorrhoea

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41
Q

Describe the management of reactive arthritis

A

Exclude septic arthritis
Aspirate and send for gram staining, culture and sensitivity plus crystal examination

NSAIDs
Intraarticular steroid injections
Oral steroids if multiple joints affected

Most resolve within 6 months and don’t recur

Recurrent cases may need DMARDs and anti-TNF medications

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42
Q

What is found in synovial fluid aspirate in reactive arthritis

A

Sterile synovial fluid with high WCC

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43
Q

Describe ankylosing spondylitis

A

Inflammatory condition affecting the spine that causes progressive stiffness and pain

Seronegative spondyloarthropathy - HLA B27 gene

Slow onset >3 months

Lower back pain and stiffness with sacroiliac pain in the buttock, pain and stiffness is worse with rest and improves with movement
Pain is worse at night and in the morning. Takes at least 30 minutes for the stiffness to improve in the morning and gets progressively better with activity throughout the day

Fluctuate with flares of worsening symptoms and other periods where symptoms improve

Vertebral fractures are a key complication

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44
Q

Which joints are affected in ankylosing spondylitis

A

Sacroiliac joints

Vertebral column joints

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45
Q

List some associated symptoms of ankylosing spondylitis

A 
Systemic - fatigue and weight loss
Chest pain - costovertebral and costosternal joints
Enthesitis - plantar fascitis and Achilles tendonitis 
Dactylitis 
Anaemia 
Anterior uveitis
Aortitis 
Hear block 
Restrictive lung disease
Pulmonary fibrosis
IBD
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46
Q

Describe a test on clinical examination to diagnose ankylosing spondylitis

A

Schober’s test

Have patient stand straight and find the L5 vertebrae - mark 10cm above and 5cm below it. Get patient to bend over and measure the distance between these points. If <20cm then this is restricted lumbar movement

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47
Q

What investigations can be done for ankylosing spondylitis

A

Inflammatory markers - CRP/ESR
HLA B27
X-ray spine and sacrum
MRI of the spine - bone marrow oedema

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48
Q

Describe the X-ray changes of ankylosing spondylitis

A

Bamboo spine
Squaring of the vertebral bodies
Subchondral sclerosis and erosion
Syndesmophyte - areas of bone growth where the ligaments insert into the bone. They occur related to the ligaments supporting the intervertebral joints
Ossification of the ligaments, discs and joints
Fusion of the facet, sacroiliac and costovertebral joints

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49
Q

Describe the management of ankylosing spondylitis

A

NSAIDs - 2-4 weeks for maximum dose then consider switching to another NSAID

Steroids - flares to control symptoms

Anti-TNF medications - etanercept or monoclonal antibody against TNF such as infliximab, adalimumab or certolizumab pegol

Secukinumab - monoclonal antibody against IL 17

Additional management - physiotherapy, exercise and mobilisation, avoid smoking, bisphosphonates to treat osteoporosis, treatment of complications, surgery is occasionally required for deformities of the spine

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50
Q

Describe systemic lupus erythematosus

A

Inflammatory autoimmune connective tissue disease

Relapsing remitting course

Chronic inflammation

Anti-nuclear antibodies - antibodies to proteins within own cells nucleus cause activation of the immune system to the body and generates an inflammatory response

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51
Q

How does SLE present

A

Non-specific symptoms

Fatigue
Weight loss
Arthralgia
Myalgia
Fever
Photosensitive malar rash - butterfly shaped rash across the nose and cheek bones which spares the nasolabial folds and gets worse with sunlight 
Lymphadenopathy and splenomegaly 
Pleuritic chest pain
SOB
Mouth ulcers
Hair loss 
Raynaud's phenomenon
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52
Q

Describe the investigations for SLE

A

Autoantibodies - ANA and anti-double stranded DNA, antiphospholipid antibodies may be seen in antiphospholipid syndrome secondary to SLE

FBC - normocytic anaemia of chronic disease

C3 and C4 levels - reduced as inflammation uses up complement

CRP/ESR - raised

Immunoglobulins - raised due to activation of B cells with inflammation

Urinalysis and urine protein: creatinine ratio for proteinuria in lupus nephritis

Renal biopsy - lupus nephritis

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53
Q

What is needed for an SLE diagnosis

A

SLICC or ACR criteria - confirm ANA presence and establish a number of clinical features suggestive of SLE

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54
Q

List some complications of SLE

A 
CVD - HTN and CAD
Anaemia of chronic disease
Infection
Pericarditis 
Pleuritic chest pain
Lupus nephritis 
Neuropsychiatric SLE - optic neuritis, transverse myelitis and psychosis
Recurrent miscarriage 
VTE
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55
Q

What is the first line treatment for SLE

A

NSAIDs
Steroids
Hydroxychloroquine (treatment of choice)
Sun cream and sun avoidance - photosensitive malar rash

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56
Q

What can be used in resistant SLE

A 
Methotrexate 
Mycophenolate mofetil
Azathioprine
Tacrolimus
Leflunomide
Ciclosporin
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57
Q

What is given to patients with severe SLE and those non responsive to other treatment

A

Biological therapies - monoclonal antibodies such as rituximab and belimumab (targets B cell activating factor)

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58
Q

What type of hypersensitivity reaction is SLE

A

Type 3 - autoimmune reaction - antigen-antibody complexes

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59
Q

Which autoantibody is most specific for SLE

A

anti-dsDNA

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60
Q

Which autoantibody is most sensitive for SLE

A

ANA

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61
Q

How does hydroxychloroquine work

A

DMARD

Anti-lysosomal - stops lysosomes from working

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62
Q

Describe discoid lupus erythematosus

A

Non-cancerous skin condition
More common in young women and darker skinned people and smokers
May develop into SLE or SCC

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63
Q

Describe the presentation of discoid lupus erythematosus

A 
Lesions on face, scalp and ears
Photosensitive 
Associated with a scarring alopecia 
Hyperpigmented or hypopigmented scars 
Appears inflamed, dry, erythematous, patchy, crusty and scaling
64
Q

Describe the management of discoid lupus erythematosus

A

Skin biopsy

Sun protection, topical steroids, intralesional steroid injections and hydroxychloroquine

65
Q

What is systemic sclerosis

A

Autoimmune inflammatory and fibrotic connective tissue disease

66
Q

What are the two types of systemic sclerosis

A

Limited cutaneous systemic sclerosis

Diffuse cutaneous systemic sclerosis

67
Q

Describe the features of limited cutaneous systemic sclerosis

A

CREST syndrome

Calcinosis
Raynaud's phenomenon 
oEsophageal dysmotility
Sclerodactyly 
Telangiectasia
68
Q

Describe the features of diffuse cutaneous systemic sclerosis

A

CREST syndrome plus cardiovascular (HTN and CAD), respiratory (pulmonary HTN and fibrosis), kidney (glomerulonephritis and scleroderma renal crisis)

69
Q

What is scleroderma

A

Hardening of the skin
Shiny, tight skin without the folds on normal skin
Most notable on hands and face

70
Q

What is sclerodactyly

A

As the skin hardens and tightens around joints it leads to a restricted range of movement and reduces function
Fat pads are lost
Skin can break and ulcerate

71
Q

Describe telangiectasia in systemic sclerosis

A

Dilated blood vessels in the skin

Fine thread appearance

72
Q

Describe calcinosis

A

Calcium deposits under the skin in the fingertips

73
Q

Describe Raynaud’s phenomenon

A

When fingertips go completely white and then blue in response to even mild cold - vasoconstriction in the vessels supplying the fingers

74
Q

What does oesophageal dysmotility in systemic sclerosis cause

A

Reflux
Oesophagitis
Swallowing difficulty

75
Q

Describe scleroderma renal crisis

A

Severe hypertension and renal failure

76
Q

Which antibodies are present in systemic sclerosis

A

ANA
Anti-centromere antibodies
Anti-scl-70 antibodies

77
Q

Which type of systemic sclerosis is anti-scl-70 antibody most associated with

A

Diffuse cutaneous systemic sclerosis

78
Q

Which type of systemic sclerosis is anti-centromere antibody associated with

A

Limited cutaneous systemic sclerosis

79
Q

What investigation is useful in Raynaud’s

A

Nailfold capillaroscopy - help to rule out systemic sclerosis

80
Q

What is seen on nailfold capillaroscopy in systemic sclerosis

A

Microhaemorrhages
Avascular areas
Abnormal capillaries

81
Q

How is systemic sclerosis diagnosed

A

Clinical features
Nailfold capillaroscopy
Antibodies

ACR and EULAR classification criteria

82
Q

How is systemic sclerosis managed

A

MDT - physio and OT

Steroids and immunosuppressants if diffuse disease and complications

No proven treatment

Non-medical management - avoid smoking, gentle skin stretching to maintain the range of motion, regular emollients, avoid cold triggers

Medical management to treat symptoms and complications - Nifedipine for Raynaud’s, antacids (PPIs) and pro-motility drugs (metoclopramide) for GI symptoms, analgesia for joint pain ,antibiotics for skin infection, antihypertensive, supportive management of pulmonary fibrosis

83
Q

What do you use to treat scleroderma renal crisis

A

ACEi (lisinopril)

84
Q

Describe polymyalgia rheumatica

A

Inflammatory condition that causes pain and stiffness in the shoulders, pelvic girdle and neck

85
Q

What condition is PMR related to

A

Giant cell arteritis

86
Q

Who does PMR usually affect

A

White women >50

87
Q

List the core features of PMR

A

> 2 weeks

Bilateral shoulder pain that might radiate to the elbow
Bilateral pelvic girdle pain
Worse with movement
Interferes with sleep
Stiffness in the morning ;lasting >45mins

88
Q

List some additional features of PMR

A

Carpal tunnel
Upper arm tenderness
Pitting oedema
Systemic symptoms - weight loss, fatigue, low grade fever and low mood

89
Q

How is a diagnose of PMR made

A

Clinical presentation
Response to steroids

Rule out other conditions

Inflammatory markers - usually raised but can be normal
FBC
U&Es
LFTs
Ca - may be raised in hyperparathyroidism and cancer or low in Osteomalacia
Serum protein electrophoresis for myeloma and other protein disorders
Thyroid stimulating hormone for thyroid function
Creatine kinase for myositis
RF for RA
Urine dipstick
Urine Bence jones protein - myeloma
CXR - lung and mediastinal abnormalities
ANA - SLE
Anti-cyclic citrullinated peptide (anti-CCP)

90
Q

Describe the treatment of PMR

A

15mg prednisolone

Review after 1 week, if no response then stop steroid and consider alternative diagnosis

Review after 3-4 weeks and if stable then think about a reducing regime of steroids 15mg till stable, 12.5mg for 3 weeks then 10mg for 4-6 weeks and then 1mg reduction every 4-8 weeks. May take 1-2 years to reduce

91
Q

Describe the Don’t STOP points for patients on long term steroids

A

Don’t - make them aware they are steroid dependent as without the steroids may risk adrenal crisis
Sick day rules - double steroids on days when ill
Treatment card - alert others they are steroid dependent in case found unresponsive
Osteoporosis prevention - bisphosphonates, vit D and calcium
Proton pump inhibitors - gastric protection

92
Q

Describe giant cell arteritis

A

Vasculitis of the medium and large arteries

93
Q

List the symptoms of GCA

A

Headache - severe, unilateral around the temple and forehead
Scalp pain
Jaw claudication
Blurred or double vision
Irreversible painless complete sight loss
Fever, muscle aches, fatigue, weight loss, loss of appetite, peripheral oedema

94
Q

How is GCA diagnosed

A

Clinical presentation
Raised ESR
Temporal artery biopsy - multinucleated giant cells

FBC - normocytic anaemia and thrombocytosis
LFTs - raised ALP
CRP usually high
Duplex ultrasound - Hypoechoic halo sign

Start steroids immediately before confirming diagnosis

95
Q

Describe the management of GCA

A

High dose steroids - 40-60mg prednisolone od (60 if visual symptoms or jaw claudication)

Review the steroids within 48hrs
Continue high dose steroids until symptoms resolved and then wean off

Aspirin 75mg OD - decreases visual loss and strokes
PPI - stomach protection with steroids

Refer to vascular for the biopsy, rheum for management and ophthalmology if visual symptoms

96
Q

What are some early complications of GCA

A

Vision loss

Stroke

97
Q

What are some late complications of GCA

A

Relapse
Steroid related effects
Stroke
Aortic aneurysm and dissection

98
Q

What are polymyositis and dermatomyositis

A

Polymyositis - autoimmune disorder where there is chronic inflammation of muscles
Dermatomyositis - autoimmune connective tissue disorder where there is inflammation of the skin and muscles

99
Q

What is a key blood test in polymyositis/dermatomyositis

A

Creatine kinase - will be elevated

100
Q

List some causes of raised creatine kinase

A 
Strenous exercise
AKI
Rhabdomyolysis
MI
Statins
101
Q

What may cause dermatomyositis or polymyositis

A

Underlying malignancy - paraneoplastic condition

102
Q

Which cancers cause dermatomyositis and polymyositis

A

Lung
Breast
Ovarian
Gastric

103
Q

Describe the presentation of polymyositis and dermatomyositis

A

Muscle pain, fatigue and weakness
Occurs bilaterally (proximal muscles)
Shoulder and pelvic girdle
Develops over weeks

Polymyositis does not have any skin features

104
Q

List the skin features found in dermatomyositis

A

Gottrons lesions - scaly red skin patches on knuckles, elbows and knees

Photosensitive rash on back, shoulders and neck

Purple rash on face and eyelids - heliotrope rash

Periorbital oedema

SC calcinosis

105
Q

Which antibodies are present in dermatomyositis

A

Anti-Mi-2 antibodies
Anti-nuclear antibodies
Anti-Jo-1 antibodies

106
Q

Which antibodies are present in polymyositis

A

Anti-Jo-1 antibodies

107
Q

How is dermatomyositis/polymyositis diagnosed

A 
Clinical presentation
Elevated CK
Autoantibodies
EMG 
Muscle biopsy - definitive diagnosis
108
Q

How is dermatomyositis/polymyositis managed

A

Corticosteroids are 1st line

Immunosuppressants (azathioprine), IV immunoglobulins and biological therapy (infliximab or etanercept) if steroids are inadequate

109
Q

What is antiphospholipid syndrome

A

Disorder with antiphospholipid antibodies where the blood becomes hypercoagulabe and the patient is prone to clotting (thrombosis an pregnancy related complications)

110
Q

What may antiphospholipid syndrome be secondary to

A

SLE

111
Q

Which antibodies are present in antiphospholipid syndrome

A

Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta2-glycoprotein 1 antibody

112
Q

List some conditions associated with antiphospholipid syndrome

A

VTE
Arterial thrombosis - stroke, MI, renal thrombosis
Pregnancy related complications - recurrent miscarriage, still birth, pre-eclampsia

Livedo reticularis - purple lace like rash that gives a mottled appearance to the skin

Libmann-sacks endocarditis - type of non-bacterial endocarditis where there are growths on the mitral valves of the heart

Thrombocytopenia

113
Q

How is antiphospholipid syndrome managed

A

Warfarin with INR 2-3 (3-4 if recurrent thrombosis)

LMWH (enoxaparin) and aspirin if pregnant

114
Q

Describe Sjogren’s syndrome

A

Autoimmune condition of the exocrine glands - symptoms of dry mucous membranes (dry mouth, eyes and vagina)

Primary Sjogren’s or secondary Sjogren’s (SLE or RA)

115
Q

What antibodies are involved in Sjogren’s syndrome

A

Anti-Ro and Anti-La

116
Q

How is Sjogren’s diagnosed on examination

A

Schirmer test <10mm

117
Q

What is the management of Sjogren’s

A

Artificial tears
Artificial salvia
Vaginal lubricants
Hydroxychloroquine may slow progression

118
Q

List some complications of Sjogren’s syndrome

A

Eye infection - conjunctivitis and corneal ulcers
Oral problems - cavities and candida
Vagina; problems - candidiasis and sexual dysfunction

Pneumonia and bronchiectasis
Non-hodgkins lymphoma
Peripheral neuropathy 
Vasculitis
Renal impaiment
119
Q

What is vasculitis and how can it be categorised

A

Inflammation of the blood vessels

Size of the vessels

120
Q

List the small vessel Vasculitides

A

Henoch Scholein purpura
Eosinophilic granulomatosis with polyangitis (churg Straus)
Microscopic polyangitis
Granulomatosis with polyangitis (Wagner’s granulomatosis)

121
Q

List the medium vasculititides

A

Granulomatosis with polyangitis
Polyarteritis nodosa
Kawasaki disease

122
Q

List the large vessel Vasculitides

A

Giant cell arteritis

Takayasu’s arteritis

123
Q

What blood test is importnant in vasculitis

A

Anti neurtrophil cytoplasmic antibody ANCA

124
Q

What are the two types of ANCA

A

pANCA and cANCA

125
Q

What is pANCA associated with

A

Microscopic polyangitis and esoinophilic granulomatosis with polyangitis

126
Q

What is c-ANCA associated with

A

Granulomatosis with polyangitis

127
Q

Describe eosinophilic granulomatosis with polyangitis (churg strauss syndrome)

A

Small and medium sized vasculitis
Lung and skin problems
Severe asthma in teenage/adulthood with raised eosinophils

128
Q

Describe microscopic polyangitis

A

Small vessel vasculitis
Renal failure
Lungs - sob and haemoptysis

129
Q

Describe granulomatosis with polyangitis (wegeners)

A

Small vessel vasculitis of the upper respiratory tract and kidneys
Epistaxis, crusty nasal secretions, hearing loss, sinusitis, saddle shaped nose due to perforated nasal septum
Lungs - cough, wheeze and haemoptysis, CXR may show consolidation

Kidney - glomerulonephritis

130
Q

Describe polyarteritis nodosa

A

Medium vessel vasculitis
Associated with hep B and c and HIV
Skin, GI tract and heart - renal impairment, strokes and MI
Associated with livedo reticularis rash

131
Q

Describe takayasus arteritis

A

Large vessel vascultiis
Affects the aorta and branches (pulmonary arteries)
From aneurysms or become narrow and blocked - pulseless disease
<40 yo and non specific symptoms with more specific symptoms of arm claudication and syncope
Diagnosed using doppler US in carotid disease or CT/MRI angiography

132
Q

Which gene is Behcet’s disease associated with

A

HLAB51

133
Q

What is Behcet’s disease

A

Inflammation
Recurrent oral and genital ulcers
Inflammation in other areas such as skin, eye, GI tract, lungs, blood vessels, MSK, CNS

134
Q

Where do Gouty tophi occur

A

DIP mainly but can occur in the elbows and ears

135
Q

List the risk factors for gout

A 
Male
Obese
High purine diet - meat
Alcohol
Diuretics
CVD/Kidney disease
FH
136
Q

Which joints are typically affected in gout

A

Base of the big toe
Wrists
Base of the thumb
Ankle and knee

137
Q

How is gout diagnosed

A

Exclude septic arthritis

Aspirated fluid will show no bacterial growth, needle shaped crystals, negatively birefringent of polarised light, monosodium urate crystals

Joint X ray

138
Q

What may be seen on an X-ray in gout

A

Joint space maintained
Lytic lesions
Punched out erosions with sclerotic borders and overhanging edges

139
Q

Describe the management of an acute flare of gout

A

NSAIDs are 1st line
Colchicine is 2nd line
Steroids can be 3rd line

140
Q

When is colchicine used in gout

A

For patients where NSAIDs are inappropriate 0- renal impairment or heart disease

141
Q

What is a common side effect of colchicine

A

Diarrhoea

142
Q

When is colchicine contraindicated

A

Blood/bone marrow disorders

143
Q

Describe the prophylaxis of gout

A

Allopurinol (xanthine oxidase inhibitor) reduces uric acid levels
Lifestyle change - lose weight, keep hydrated, minimise consumption of alcohol and purine foods

144
Q

When should you start gout prophylaxisis

A

When the acute attack has settled

145
Q

What so you do with the allopurinol if a patient has another attack

A

Continue them on this during the attack

146
Q

Describe pseudogout

A

Crystal arthropathy
Calcium pyrophosphate crystals
Chondrocalcinosis
Acutely hot, swollen, stiff and painful knee, shoulder, wrist or hip

147
Q

How is pseudogout diagnosed

A

Aspirated fluid shows no bacterial growth, calcium pyrophosphate crystals, rhomboid shaped, positively birefringent of polarised light

Chondrocalcinosis (white line in middle of joint space) on XRAY

148
Q

How is pseudogout managed

A 
NSAIDs
Colchicine
Joint aspiration
Steroid injection
Oral steroids 
joint washout in severe cases
149
Q

What is osteomalacia

A

Defective bone mineralisation - soft bones
Insuffieicnt vit D
Weak bones, muscle weakness, bone pain and fractures
Seocndary hyperparathyroidism

150
Q

What are the investigations of Osteomalacia

A 
Serum 25-hydroxyvitamin D <25
Serum calcium low 
Serum phosphate low
Serum ALP may be high 
PTH may be high 
Xray - osteopenia 
DEXA - reduced BMD
151
Q

How is Osteomalacia treated

A

Supplementary vitamin D - colecalciferol 50,000IU once weekly for 6 weeks

152
Q

What is Pagets disease of bone

A

Disorder of bone turnover - excessive activity of osteoblasts and osteoclasts
Sclerosis and lysis of bone also bone enlargement and deformity
Axial skeleton
Increased risk of pathological fractures

153
Q

List some Xray changes in pagets disease of bone

A

Bone enlargement and deformity
Osteoposiris circumscripta - osteolytic lesions less dense than normal bone
Cotton wool appearance of the skull - decreased density and increased densoity
V sahped defects in long bones

154
Q

What biochemical test will be abnormal in pagets disease of bone

A

ALP - raised

155
Q

How is pagets disease of bone treated

A

Bisphosphonates - interfere with osteoclast activity

NSAIDs - pain
Calcium and vit D supplementation
Surgery

156
Q

List 2 complications of pagets disease

A

Osteosarcoma

Spinal stenosis

Finals (18 decks)

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