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Phase 4 Question Knowledge > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

RA

A
  • Chronic autoimmune inflammation of synovial joints
  • inflammation of synovial lining, tendon sheaths, bursae
  • symmetrical polyarthritis - 5+ joints affected
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2
Q

RA Px

A
  • joint pain
  • stiffness - >30mins in morning, better with activity
  • inflammation - red, hot, swollen
  • fatigue, malaise, wt loss, myalgia
  • symmetrical distal polyarthritis - small joints of hands / feet
  • ankle, knee, hips, shoulders, c-spine
  • atlanto-axial subluxation

Hand sx

  • wrist, MCP, PIP, MTP joints affected
  • swan neck (flexed DIP)
  • Boutonniere (flexed PIP)
  • z thumb
  • ulnar deviation
  • subluxation
  • positive squeeze test - MCP/MTP joints

Extra-articular sx

  • pulm fibrosis
  • Felty’s syndrome - RA + neutropenia + splenomegaly
  • Sjogren’s
  • anaemia chronic disease
  • CV disease
  • rheumatoid nodules
  • lymphadenopathy
  • CTS
  • amyloidosis
  • bronchiolitis obliterans
  • Caplan syndrome - pulmonary nodules

Eye sx

  • Dry eye syndrome – keratoconjunctivitis sicca
  • episcleritis, scleritis, keratitis
  • cataracts (steroids)
  • retinopathy
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3
Q

RA Ix

A
  • FBC, CRP/ESR, RF, anti-CCP
  • XR - LESS - loss of joint space, erosions (bony), soft tissue swelling, soft bones
  • US / MRI - if synovitis unclear
  • HAQ / DAS28 scoring systems
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4
Q

RA Mx

A
  • physio, podiatry
  • DAS28, CRP to monitor tx
  • paracetamol, NSAIDs
  • prednisolone - at initial px / starting new tx / flares
  • DMARDs - methotrexate, sulfasalazine, leflunomide, hydroxychloroquine
  • biological therapy - infliximab / etanercept (TNF inhibitors), rituximab (anti-CD20)
  • surgery
  • pregnancy - hydroxychloroquine / sulfasalazine + folic acid
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5
Q

Psoriatic arthritis

A
  • inflammatory arthritis with psoriasis
  • HLAB27, RF-

5 patterns of disease

  • Asymmetrical oligoarthritis – 1-4 joints, one side of body
  • Symmetrical polyarthritis – like RA
  • DIP predominant pattern – primarily DIP joints affected
  • Spondylitis – back stiffness, pain – axial skeleton – spine + SI joints
  • Arthritis mutilans – phalanges – osteolysis (destruction), shortening of digits – telescoping digit
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6
Q

PsA Px

A
  • joint pain, swelling, stiffness
  • psoriatic plaques on skin - knees, elbows, behind ear, scalp
  • nail pitting
  • onycholysis
  • dactylitis
  • enthesitis
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7
Q

PsA Ix

A
  • bloods - RF-, high ESR/CRP
  • Psoriasis Epidemiological Screening Tool (PEST)
  • XR - erosions central in joint (pencil in cup deformity), periostitis, ankylosis, osteolysis, dactylitis
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8
Q

PsA Mx

A
  • physio, exercise, surgery
  • NSAIDs
  • steroids - can be intra-articular
  • DMARDs
  • anti-TNF
  • ustekinumab
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9
Q

Reactive arthritis

A
  • joint inflammation in autoimmune response to infection
  • HLAB27
  • Post-STI - SARA - sexually acquired reactive arthritis
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10
Q

ReA Px

A
  • <4wks of infection, may last 4-6mo
  • asymmetrical joint pain, warm, red, stiff, swollen
  • malaise, fatigue, fever
  • dactylitis
  • mouth ulcers
  • Reiter’s - bl conjunctivitis, anterior uveitis, urethritis, enthesitis / arthritis
  • skin changes, nail dystrophy
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11
Q

ReA Ix

A
  • bloods - ESR / CRP raised
  • STI screen, GUM referral
  • stool culture
  • XR - enthesitis
  • joint aspiration - MC+S, crystal examination
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12
Q

ReA Mx

A
  • NSAIDs
  • steroid injection
  • tx infection
  • if sx >6mo - DMARDs, anti-TNF
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13
Q

Ankylosing spondylitis

A
  • inflammatory arthritis of axial skeleton - spine + SI joints
  • HLAB27
  • enthesitis inflammation, syndesmophyte formation, fibrosis + ossification, ankylosis
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14
Q

AS Px

A
  • lover back pain, stiffness
  • pain at night, worse in morning, >30mins, improves with movt
  • peripheral arthritis 1-2 joints
  • kyphosis

Extraarticular features

  • Anterior uveitis
  • Amyloidosis
  • Apical lung fibrosis
  • Aortic regurg
  • AV node block
  • Achilles tendonitis
  • Anaemia of chronic disease

SPINEACHE

  • Sausage digit (dactylitis)
  • Psoriasis
  • Inflammatory back pain
  • NSAID good response
  • Enthesitis (heel)
  • Arthritis
  • Crohn’s/colitis/elevated CRP (but can be normal)
  • HLAB27
  • Eye (uveitis)
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15
Q

AS Ix

A
  • Schober’s - reduced spinal flexion
  • Bloods - raised ESR/CRP, anaemia, HLAB27+
  • spirometry - restrictive
  • XR spine/sacrum - sacroiliitis, fusion of joints, ossification, subchondral sclerosis, squaring of vertebrae, bamboo spine, syndesmophites
  • CXR - apical fibrosis
  • MRI - bone marrow oedema
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16
Q

AS Mx

A
  • Exercise, OT/PT, stop smoking
  • NSAIDs
  • DMARDs
  • Anti-TNF, MAbs, JAK inhibitor
  • intra-articular steroids
  • surgery - joint deformity
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17
Q

Osteomalacia

A
  • soft bones - decreased bone mineral content + low vit D
  • uncalcified osteoid + cartilage
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18
Q

Osteomalacia causes

A
  • vit D deficiency
  • CKD
  • Drugs - AEDs
  • liver cirrhosis
  • tumour induced
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19
Q

Osteomalacia Px

A
  • bone pain
    bone / muscle tenderness
  • fractures - eg femoral neck
  • proximal myopathy - waddling gait
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20
Q

Osteomalacia Ix

A
  • bloods - low 25-OH vit D (low 1,25-OH vit D in renal failure), low Ca, phos, raised ALP, high PTH
  • XR - translucent bands - Looser’s zones
  • DEXA scan - low bone mineral density
  • (biopsy)
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21
Q

Osteomalacia Mx

A
  • vit D supplementation - colecalciferol / calcitriol
  • Ca supplementation
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22
Q

Osteoporosis

A
  • low bone mass -> fragile bones, fractures
  • osteopenia - less severe, reduced bone mass
  • hip, vertebral, colles - common
  • post-menopause - lack of oestrogen -> increased bone turnover (resorption>formation)
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23
Q

Osteoporosis RFs

A
  • older
  • female
  • post-menopause
  • reduced mobility / activity
  • low BMI
  • low Ca / vit D intake
  • alcohol, smoking
  • Hx / FHx fractures
  • chronic disease - CKD, hyperthyroid, RA, Cushing’s
  • testosterone deficiency - hypogonadism
  • long-term steroids
  • Meds - SSRIs, PPIs, AEDs, anti-oestrogens, Depo-provera
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24
Q

Osteoporosis Px

A
  • asym
  • increased fractures
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25
Osteoporosis Ix
- FRAX - QFracture Tool - DEXA scan (femoral neck) - >-1 normal, -1 to -2.5 osteopenia, <-2.5 osteoporosis, +# is severe osteoporosis - XR - for secondary causes - FBC, U/E, CRP/ESR, bone profile, TFT, LFTs - >75yo with fragility# + suspected osteoporosis - start on bisphosphonate (<75yo, will need FRAX / DEXA)
26
Osteoporosis Mx
- healthy weight, exercise, stop smoking, reduce alcohol - adequate vit D / Ca intake - Tx if T<-2.5 - bisphosphonates - alendronate - denosumab - teriparatide - HRT - raloxifene - strontium ranelate - >75yo with fragility# + suspected osteoporosis - start on bisphosphonate (<75yo, will need FRAX / DEXA)
27
Paget's disease of bone
- focal bone remodelling due to increased bone turnover - increased osteoclast/blast activity, new bone weaker, fibrous - skull, spine/pelvis, femurs
28
Paget's Px
- asym 60-80% - bone pain - bowing of tibia, bossing of skull
29
Paget's Ix
- Bloods - raised ALP - raised urinary hydroxyproline - XR - local bony enlargement, sclerotic changes, osteolytic areas, skull (thickened vault, osteolytic lesions, cotton wool appearance) - bone scintigraphy
30
Paget's Mx
- bisphosphonates - calcitonin - analgesia - NSAIDs - Ca / vit D supplements - surgery - fracture / deformity - monitor with ALP / sx
31
Gout
- joint inflammation due to deposition of monosodium urate (MSU) crystals in joint - high serum uric acid levels (from purines)
32
Gout causes
Decreased excretion - drugs - thiazides - CKD - lead toxicity - alcohol - obesity - DM Increased production - high purine diet - meat, seafood - hyperlipidaemia - alcohol .... RFs - male, FHx, CVD
33
Gout Px
- acute flare episodes - pain - swelling - erythema - most 1st MTPJ, also 1st MCPJ, ankle, wrist, knee - gouty tophi - hands, elbows, ears
34
Gout Ix
- serum uric acid - Joint aspiration - MC+S, polarised light microscopy - needle shaped negatively birefringent crystals - XR - joint effusion, punched out erosions, sclerotic margins, lytic lesions in bone, joint space preserved
35
Gout Mx
Acute flares - NSAIDs (+PPI) - colchicine - oral steroids - prednisolone - consider intra-articular steroids Prophylaxis - lose weight, hydrate, minimise purine intake, alcohol - allopurinol - febuxostat - don't start until >4wks after attack, continue during future attacks
36
Pseudogout
- acute inflammation of joint from deposition of calcium pyrophosphate (CPP) crystals - >65yo - in younger pts - HHC, hyperparathyroid, low Mg, low phos, acromegaly, Wilson's
37
Pseudogout Px
- asym - pain, stiffness, swelling, hot joint - knee, shoulder, hip, wrist
38
Pseudogout Ix
- joint aspiration - MC+S, polarised light microscopy - positively birefringent rhomboids - XR - chondrocalcinosis, loss of joint space, osteophytes, sclerosis, cysts
39
Pseudogout Mx
- NSAIDs + PPI - colchicine - intra-articular steroid injection / oral steroids
40
Fibromyalgia
- syndrome of widespread chronic pain, specific tenderness
41
Fibromyalgia Px
- chronic pain - specific sites / all over - lethargy - cognitive impairment - fibro fog - sleep disturbance, headaches, dizziness
42
Fibromyalgia Ix
- clinical dx - America College of Rheumatology - criteria - lists 18 tender points - if tender in 11/18, dx more likely
43
Fibromyalgia Mx
- Educate about condition - Aerobic exercise - CBT - Pregabalin, duloxetine, amitriptyline
44
Systemic lupus erythematosus (SLE)
- inflammatory autoimmune multi-system disorder
45
SLE Patho
- relapsing-remitting - flares of worse sx - Anti-nuclear ABs (ANA) - auto-ABs against cell nucleus -> chronic inflammatory response - T3 hypersensitivity - immune complex deposition - flare triggers - sun, oestrogen, pregnancy, drugs, infection, stress Drug-induced lupus - eg procainamide, hydralazine, isoniazid, phenytoin - arthralgia, myalgia, malar rash, pleurisy
46
SLE Px
General - Fatigue - Weight loss - Fever - Lymphadenopathy - Mouth ulcers - Splenomegaly Skin - Photosensitive malar rash - Raynaud’s - Hair loss - Livedo reticularis - Discoid rash MSK - arthralgia (polyarticular), Jacoud's arthropathy - arthritis - myalgia CV - pericarditis - myocarditis Resp - SOB - pleuritic CP Renal - proteinuria - glomerulonephritis / oedema Neuropsych - anxiety, depression - psychosis - seizures
47
SLE Ix
- auto-AB screen - ANA, anti-dsDNA, anti-Sm - FBC - anaemia, low WCC/platelets - low C3/4 - urinalysis, urine protein:creatinine ratio - renal biopsy
48
SLE Mx
- sunscreen / avoid sun - NSAIDs - hydroxychloroquine - steroids - prednisolone - DMARDs - biological - rituximab, belimumab
49
SLE Cx
- CV disease - Infection - Anaemia - Pericarditis - Pleuritis - Interstitial lung disease - Lupus nephritis - Neuropsychiatric SLE - inflammation in CNS - optic neuritis, transverse myelitis, psychosis - Recurrent miscarriage - VTE
50
Discoid lupus erythematosus
- autoimmune chronic skin condition - follicular keratin plugs - <5% progress to SLE Px - erythematous, raised rash, +/- scaly - photosensitive - face, neck, ears, scalp - lesions heal with atrophy, scarring, pigmentation, alopecia Ix - skin biopsy Mx - topical steroid cream - hydroxychloroquine - avoid sun exposure
51
Systemic sclerosis
- autoimmune disorder - inflammation + fibrosis of connective tissues - increased fibroblast activity + collagen deposition Scleroderma - hardening of skin Limited cutaneous SSc - CREST Diffuse cutaneous SSc - CREST and internal organs
52
SSc Px
Scleroderma - hardening of skin - shiny/tight Limited - CREST - calcinosis - Raynaud's - (o)esophageal dysmotility - reflux/dysphagia - sclerodactyly - telangiectasia Diffuse - CV - HTN, coronary heart disease, arrhythmias - Lung - pulm HTN, pulm fibrosis - dry cough, SOB - Kidney - glomerulonephritis, scleroderma renal crisis (HTN, renal failure)
53
SSc Ix
- bloods - anaemia, raised ESR, auto-ABs - anti-centromere (limited), anti-Scl-70 (diffuse), RF+, ANA+ - urinalysis - AKI, proteinuria - CXR, hand XR, barium swallow, HRCT - nailfold capillaroscopy
54
SSC Mx
- Mx raynauds - stop smoking, emollients, OT/PT - PPI, analgesia, abx, ACEi, - bosentan, sildenafil - IV iloprost - digital ulcers - cyclophosphamide / pred - stem cell transplant
55
Raynaud's
- episodic vasospasm in fingers (rarely toes) in response to cold/stress Primary - Raynaud's disease Secondary - scleroderma, RA, SLE - leukaemia, cryoglobulinaemia - vibrating tools - OCP - cervical rib Factors suggesting connective tissue disease - >40yo, unilateral, rashes, auto-ABs, sx of RA / SLE, digital ulcers, calcinosis, abnormal nail-fold capillaries
56
Raynaud's Px
- white -> blue -> red discolouration of fingers - ischaemia -> deoxygenation -> reactive hyperaemia - usually bilateral
57
Raynaud's Mx
- secondary care referral if <12yo, ?secondary, poor sx control - keep body warm, stop smoking, exercise - nifedipine - IV prostacyclin - losartan, ACEi, sildenafil, fluoxetine - admit if severe ischaemia
58
Sjogren's syndrome
- autoimmune destruction of exocrine glands - primary / secondary (RA, SLE, SSc) - lymphocytic infiltration / fibrosis of glands
59
Sjogren's Px
- dry eyes - blurry, itchy, red, burning - dry mouth, caries - cracks/fissures, difficulty swallowing, dry cough - parotid swelling - nasal septum crusting/bleeding - vaginal dryness, dyspareunia - systemic signs - polyarthritis, Raynaud's, lymphadenopathy, fatigue, vasculitis
60
Sjogren's Ix
- Bloods - anti-Ro, anti-La ABs, RF+ 50%, ANA+ 70% - Schirmer's test
61
Sjogren's Mx
- artificial tears - artificial saliva - vaginal lubricants - oral pilocarpine - hydroxychloroquine (consider)
62
Myositis
- autoimmune inflammation / necrosis of muscles - polymyositis - muscle proteins attacked - dermatomyositis - muscle, skin, other organs attacked - malignancy association, also Coxsackie, HIV
63
Myositis Px
Polymyositis - symmetrical progressive proximal muscle weakness - shoulders, hips - dysphagia, dysphonia, resp failure - fatigue, myalgia, muscle cramps - hand fine motor movts affected Dermatomyositis - as above - heliotrope - periorbital oedema - Gottron's sign - photosensitive erythematous rash on back, shoulders, neck - GI ulcers, infections Extra-muscular sx - fever, arthralgia, Raynaud's, lung fibrosis
64
Myositis Ix
- bloods - elevated CK, LDH, aldolase, AST, ALT - auto-ABs - anti-Jo-1, ANA+ 80% - EMG - MRI, CXR, PFTs, HRCT - muscle biopsy
65
Myositis Mx
- OT/PT - oral prednisolone - immunosuppressants - methotrexate, azathioprine - IV Ig - biologics - infliximab, etanercept
66
Behcet's disease
- complex autoimmune inflammatory condition affecting vessels / tissues
67
Behcet's Px
- oral ulcers + genital ulcers + anterior uveitis - thrombophlebitis, DVT, aneurysms - arthritis - Neuro - aseptic meningitis, cerebral venous sinus thrombosis - GI - abdo pain, diarrhoea, colitis - Skin - erythema nodosum, papules / pustules like acne - Sx vary, van be relapsing-remitting
68
Behcet's Ix
- clinical dx - positive pathergy test
69
Behcet's Mx
- soluble betamethasone tablets - mouth ulcers - oral prednisolone - colchicine - lidocaine ointment - genital ulcers - immunosuppressants - azathioprine - biologic - infliximab
70
Chronic fatigue syndrome (CFS)
- Disabling fatigue affecting physical / mental function >50% of the time, in the absence of other disease explaining sx
71
CFS Px
- Fatigue (suspect after >6wks), >50% of the time - Sleep problems - Muscle / joint pains - Headaches - Painful lymph nodes, without enlargement - Sore throat - Cognitive dysfunction - difficulty thinking, memory impairment - Sx worse with physical / mental exertion - Malaise, flu-like sx - Dizziness - Nausea - Palpitations
72
CFS Ix
- Bloods to r/o other causes - FBC, U/E, LFTs, glucose, HbA1c, TFT, ESR, CRP, Ca, phosph, CK, ferritin, coeliac screen, urinalysis - clinical dx - sx >3mo
73
CFS Mx
- refer to CFS service - energy management - physical exercise / activity - CBT
74
Vasculitis
- inflammation of blood vessels - also necrosis, vessel wall destruction, thrombosis, ischaemia - anaemia, raised ESR Classification Large vessel - GCA/temporal arteritis/PMR - Takayasu's arteritis Medium vessel - polyarteritis nodosa - Kawasaki's Small vessel ANCA associated - microscopic polyangiitis (p-ANCA - perinuclear) - granulomatosis with polyangiitis (c-ANCA - cytoplasmic) - eosinophilic granulomatosis with polyangiitis (p-ANCA) Immune complex - essential cryoglobulinaemia - HSP - Goodpasture's - cutaneous leucocytic vasculitis
75
Vasculitis general Px
- Joint / muscle pain - Peripheral neuropathy - Renal impairment - Purpura - Necrotic skin ulcers - GI sx - diarrhoea, abdo pain, bleeding - Fatigue - Fever - Night sweats - Wt loss - Anorexia - Anaemia
76
Vasculitis general Ix
- CRP / ESR raised - check ANCA
77
Vasculitis general Mx
- steroids - cyclophosphamide / rituximab
78
Polymyalgia rheumatica (PMR)
- inflammatory condition causing pain/stiffness in shoulders, pelvic girdle, neck - GCA associated
79
PMR Px
- rapid onset days/wks - sx present >2wks for PMR dx - pain / stiffness - shoulders, pelvic girdle, neck - worse in morning/after rest - sleep interference - >45mins morning stiffness - systemic - wt loss, fatigue, fever - muscle tenderness - CTS - peripheral oedema
80
PMR Ix
- raised ESR/CRP - bloods to r/o ddx - FBC, U/E, LFTs, Ca, serum protein electrophoresis, TFTs, CK, RF, urine dip - consider - ANA, anti-CCP, urine bence-jones, CXR
81
PMR Mx
- oral prednisolone 15mg - reducing regime (may last 1-2yrs) Long term steroid mx - dont STOP Don't - stop abruptly S - sick day rules - increase dose when unwell T - Tx card O - osteoporosis - bisphosphonates, Ca, vit D P - PPI - omeprazole
82
Temporal arteritis / GCA
- vasculitis of medium/large arteries - 50% of GCA pts will have PMR - ocular cx - from anterior ischaemic optic neuropathy - occlusion of posterior ciliary artery (branch of ophthalmic)
83
GCA Px
- onset <1mo - unilateral headache - jaw claudication - amaurosis fugax - scalp tenderness - tender / thickened artery - PMR sx - lethargy, depression, fever, anorexia, night sweats - muscle tenderness, CTS, peripheral oedema
84
GCA Ix
- raised ESR, maybe CRP - fundoscopy - temporal artery biopsy - skip lesions, multinucleated cells - Duplex USS
85
GCA Mx
- steroids - prednisolone / IV methylpred - wean over 1-2yrs - ophthal / rheum referrals, maybe vascular - bisphosphonates, vit D, Ca, PPI - low dose aspirin
86
Takayasu's arteritis
- large vessel vasculitis - aorta, branches, pulmonary arteries - vessels swell - aneurysms / narrow + block Px - <40yo - fever, malaise, muscle aches - claudication sx - upper/lower limbs - unequal BP in limbs - carotid bruit / tenderness - absent / weak peripheral pulses - aortic regurg 20% Ix - CT / MRI angiography Mx - steroids
87
Polyarteritis nodosa (PAN)
- medium-vessel vasculitis, necrotising inflammation, aneurysms - associated with hep B Px - fever, malaise, arthralgia, wt loss, HTN - mononeuritis multiplex - testicular pain, livedo reticularis, haematuria, renal failure - MI, stroke, mesenteric arteritis (GI pain), tender, erythematous skin nodules Ix - pANCA (20%), hep B serology, anaemia, raised WCC/ESR - Biopsy - eg kidney - angiography Mx - ACEi - prednisolone - immunosuppression - azathioprine / cyclophosphamide - tx HBV - antivirals
88
ANCA-associated vasculitis
Anti-neutrophil cytoplasmic ABs (ANCA), associated with - Microscopic polyangiitis – pANCA – perinuclear - Granulomatosis with polyangiitis – cANCA – cytoplasmic - Eosinophilic granulomatosis with polyangiitis – pANCA Common features - Renal – immune complex glomerulonephritis -> raised creatinine, haematuria, proteinuria - Resp – SOB, haemoptysis - Systemic – fatigue, fever, wt loss - Vasculitic rash – in minority - ENT – sinusitis General Ix - urinalysis - bloods - FBC, CRP, ANCA - CXR General Mx - renal / rheum / resp referral - kidney / lung biopsies - immunosuppression
89
Microscopic polyangiitis
- small vessel vasculitis - glomerulonephritis -> renal failure - raised creat, haematuria, proteinuria - lungs - diffuse alveolar haemorrhage, haemoptysis, cough, SOB - fever, lethargy, myalgia, wt loss - mononeuritis multiplex - pANCA in 50-75%, cANCA 40%
90
Granulomatosis with polyangiitis
- Wegener's granulomatosis - small vessel vasculitis affecting lungs + kidneys Px - epistaxis, nose crusting - saddle-shaped nose - sinusitis - hearing loss - cough, wheeze, haemoptysis - glomerulonephritis - vasculitic rash, proptosis, CN lesions Ix - cANCA+ - CXR - renal biopsy Mx - steroids - cyclophosphamide - plasma exchange
91
Eosinophilic granulomatosis with polyangiitis
- Churg-Strauss syndrome - small vessel vasculitis affecting lungs, skin, kidneys... Px - severe asthma - sinusitis, allergic rhinitis - mononeuritis multiplex Ix - FBC - raised eosinophils
92
Antisynthetase syndrome
Syndrome caused by anti-Jo1 autoABs Characterised by - Myositis - ILD - Thickened / cracked skin of hands - Raynaud's
93
Hypersensitivity
Type 1 - anaphylactic - Ag reacts with IgE bound to mast cells - anaphylaxis, atopy Type 2 - cell bound - IgM / IgG binds to Ag on cell surface - AIHA, ITP, Goodpasture's, pernicious anaemia, acute haemolytic transfusion reaction, rheumatic fever Type 3 - immune complex - free Ag and Ab (IgG/IgA) combine - SLE, post-strep glomerulonephritis, EAA Type 4 - delayed hypersensitivity - T cell mediated - TB, graft vs host disease, scabies... (Type 5 - ABs bind to cell receptors) - Graves, MG
94
Langerhans cells histiocytes
Abnormal proliferation of histiocytes – childhood, bony lesions Px - Bone pain, typically in the skull or proximal femur - Cutaneous nodules - Recurrent otitis media/mastoiditis - Tennis racket-shaped Birbeck granules on electromicroscopy
95
McArdle's disease
- autosomal recessive type V glycogen storage disease - myophosphorylase deficiency -> decreased muscle glycogenolysis Px - Muscle pain, stiffness after exercise - Muscle cramps - Myoglobulinuria - Low lactate during exercise
96
Still's disease in adults
- Bimodal age distribution – 15-25yo, 35-46yo Px - arthralgia - salmon pink, maculopapular rash - fever - lymphadenopathy Ix - raised serum ferritin - RF / ANA - - Yamaguchi criteria to dx Mx - NSAIDs - Steroids - consider methotrexate, biologics

Phase 4 Question Knowledge (36 decks)

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