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ALS > Rheum > Flashcards

Rheum Flashcards

1
Q

Temporal Arteritis

A

Medium/ large vessel vasculitis, 50% have PMR

= >60yrs, <1mth onset, headache, jaw claudication, tender palpable artery, PMR symptoms

Inv - ^ESR/CRP, normal CK, normal EMG, temporal artery biopsy (skip lesions), vision testing

-> urgent high dose steroids (before biopsy), pred vs methylpred and urgent opth review if vision loss

Comp - anterior ischaemic optic neuropathy (swollen pale disc, blurred margins), amaurosis fugax or permanent

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2
Q

Polymyalgia Rheumatica

A

= >60yrs, morning stiffness in prox muscles, aching, no true weakness

Inv - ^ESR, normal CK, normal EMG

-> pred, reconsider if no drastic response

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3
Q

ANCA Vasculitis

A

cANCA - Granulomatosis with polyangiitis

pANCA - Eosinophilic granulomatosis with polyangiitis, Microscopic polyangiitis, UC, PSC, anti-GBM

= immune complex GN, SOB, hemoptysis, fatigue, fever, weight loss, rash, sinusitis

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4
Q

Ankylosing Spondylitis

A

HLA B27 associated spondyloarthropathy

= 20-30yrs, M, lower back pain, stiffness, better with exercise, v lateral and forward flexion (Schober’s), v chest expansion

Extra articular features: As
Apical fibrosis
Anterior Uveitis
Achilles tendinopathy
AV node block
Aortic regurgitation
Amyloidosis
Arthritis (peripheral, ^F)
And cauda equina

Inv - ^ESR/ CRP, XR spine shows sacroiliitis (subchondral erosion, sclerosis), squaring of lumbar vertebrae, bamboo spine, syndesmophytes, spirometry (restrictive)

-> regular exercise, NSAIDs, physio, anti-TNF if persistent

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5
Q

Behcet’s Syndrome

A

Multisystem AI-mediated inflammation of arteries and veins

RF - 20-40yrs, M, east Mediterranean, HLA B51, FHx

= oral ulcers, genital ulcers, anterior uveitis, GI, thrombophlebitis, DVT, arthritis, erythema nodosum

Inv - clinical, pathergy test

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6
Q

Bone Disorders: Labs

A

Ca, PO4, ALP, PTH

Osteoporosis - normal, normal, normal, normal
*check testosterone in men

Osteomalacia - low, low, high, high

Primary hyperparathyroidism - high, low, high, high

Secondary (CKD) - low, high, high, high

Page’s - normal, normal, high, normal

Osteopetrosis - normal, normal, normal, normal

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7
Q

Chronic Fatigue Syndrome/ ME

A

> 3m disabling fatigue, affects mental and physical function, no link to psych Hx

= F, fatigue, worse on activity, post-exertional malaise, sleep issues, cog dysfunction

Inv - excl pathology (FBC, U&E, LFT, glucose, TFT, CRP, calcium, CK, ferritin, coeliac, urine dip)

-> refer to CFS specialist, energy management, CBT

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8
Q

Polymyositis

A

Inflammatory disorder causing symmetrical proximal muscle weakness

Link - malignancy

= prox muscle weakness, tenderness, resp weakness, lung disease

Inv - ^CK, ^LDH/ AST/ ALT, EMG, muscle biopsy, anti-Jo1

-> high dose steroids

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9
Q

Dermatomyositis

A

Inflammatory disorder causing symmetrical proximal muscle weakness and skin lesions

Link - underlying cancer (ovarian, breast, lung), CTD

= photosensitivity, macular rash on back/ shoulders, periorbital heliotrope rash, Gottron’s (rough red papules, finger extensors), mechanic’s hands, nailfold cap dilatation

Inv - ANA (80%), anti-synthetase Ab (anti Jo-1, Mi-2)

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10
Q

SLE

A

Multisystem AI disorder, T3 HS reaction (immune complex)

RF - F, Afro-Caribbean/ Asian, HLA DR2/3

= fatigue, fever, mouth ulcers, nodes

Skin = malar rash (spares nasolabial folds), red scaly discoid rash (sun exposed), photosensitivity, Raynaud’s, livedo reticularis, alopecia

MSK = arthralgia, non-erosive arthritis

CV = pericarditis, myocarditis

Renal = diffuse proliferative GN, proteinuria

Neuropsych = anxiety, depression, psychosis, seizures

Inv - ANA (99%, sensitive), anti- dsDNA (specific), anti-smith, RF (20%), ^ESR, v C3/ 4 in active phase

-> hydroxychloroquine, NSAIDs, steroids for organs

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11
Q

Discoid Lupus Erythematous

A

Benign AI disorder, <5% progress to SLE

= erythematous raised rash on head, may be scaly, photosensitive, scarring/ pigmentation

-> top steroid, hydroxychloroquine 2nd

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12
Q

Drug-Induced Lupus

A

= arthralgia, myalgia, malar rash, pleurisy (less renal/ nervous)

Cause - procainamide, hydralazine, isoniazid, phenytoin

Inv - ANA (100%), dsDNA negative, anti-histone Ab

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13
Q

Fibromyalgia

A

= F, 30-50yrs, widespread pain, tender points, lethargy, fibro fog

Inv - 9 pairs of tender points (likely if 11/18)

-> explain, aerobic exercise, CBT

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14
Q

Gout

A

Inflammatory arthritis, ^1st MTP (or ankle, wrist, knee)

RF - thiazide diuretics, CKD, myeloproliferative disorder, severe psoriasis, cytotoxic drugs

= pain, swelling, redness, flares last days

Inv - uric acid, synovial fluid analysis (-ve birefringent needle-shaped crystals), XR (effusion, JA punched-out erosions, overhanging edges, preservation of joint space, soft tissue tophi)

-> NSAIDs/ colchicine (SE: diarrhea), steroid if not, allopurinol long term (continue during flare, start with colchicine cover), v alcohol, weight loss, v purines

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15
Q

Langerhans Cell Histiocytosis

A

Rare, abnormal proliferation of histiocytes

= child, bone pain, skull (punched out, beveled), proximal femur, cutaneous nodules, recurrent OM/ mastoiditis

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16
Q

McArdle’s Disease

A

AR, type V glycogen storage disease, v muscle glycogenolysis

= muscle pain and stiffness post-exercise, cramps

Inv - myoglobinuria, v lactate during exercise

17
Q

Osteogenesis Imperfecta

A

AD, type 1 collagen disorder (brittle bone disease)

= child, fractures from minor trauma, blue sclera, deafness (otosclerosis), dental issues

Inv - normal bone profile

18
Q

Osteomalacia

A

Softening of bones 2nd to v Vit D, rickets in kids

Cause - malabsorption, lack of sunlight, diet, CKD, liver cirrhosis, coeliac disease, antiepileptics

= bone pain, bone/ muscle tenderness, fractures (femoral neck), proximal myopathy (waddling gait)

Inv - v Ca/PO4, ^ALP, ^PTH, XR (translucent bands)

-> vit D supplementation, calcium if diet inadequate

19
Q

Polyarteritis Nodosa

A

Medium vessel vasculitis, necrotising inflammation and microaneurysms

RF - middle-age M, hep B

= fever, malaise, joint pain, weight loss, HTN, mononeuritis multiplex, teste pain, livedo reticularis, haematuria

Inv - hep B serology (30%), pANCA (20%)

20
Q

Pseudogout

A

Deposition of calcium pyrophosphate crystals in synovium

RF - ^age, haemochromatosis, ^PTH, v Mg/ PO4, Wilson’s, acromegaly

= ^knee, wrist, shoulders

Inv - joint aspiration (+ve bf rhomboid crystals), XR (chondrocalcinosis)

-> NSAIDs, IA/ PO steroids

21
Q

Psoriatic Arthrtis

A

Inflammatory arthritis, HLA B27 seroneg

Patterns
- symmetrical poly, asymmetrical oligo (hands/ feet), sacroiliitis, DIP joint disease, arthritis mutilans (telescope fingers)

= may precede skin lesions, nail pitting, onycholysis, tenosynovitis, enthesitis (Achilles, plantar), dactylitis

Inv - XR (erosion and new bone, pencil in cup, periostitis)

-> refer to rheum, NSAIDs, DMARD if mod/severe, mAb

22
Q

Raynaud’s

A

Exaggerated vasoconstrictive response of digital arteries and cutaneous arterioles to cold or emotional distress

Cause - primary or 2nd to scleroderma, RA, SLE, leukaemia, cold agglutinins, cryoglob, vibrating tools

-> refer all if suspect 2nd, nifedipine (CCB), IV prostacyclin

23
Q

Reactive Arthritis

A

HLA B27 seroneg, last <12mths

Cause - 4wks post-STI (^M, chlamydia) or dysentery (shigella, salmonella, campylobacter)

= urethritis, conjunctivitis, arthritis (asym, oligo), circinate balanitis, keratoderma blennorrhagica, dactylitis, uveitis

-> NSAID, IA steroid, sulfasalazine/ methotrexate if persist

24
Q

RA - Features

A

= swollen painful joints, hand and feet then larger joints, stiff in morning, positive squeeze test, swan neck, boutonniere

Comp

Lung - pulmonary fibrosis, pleural effusion, pulmonary nodules, bronchiolitis obliterans, methotrexate pneumonitis, pleurisy

Eyes - keratoconjunctivitis sicca, episcleritis, scleritis, corneal ulceration, keratitis, steroid-induced cataracts, chloroquine retinopathy

Others - OP, IHD (same risk at T2DM), depression, Felty’s syndrome (RA + splenomegaly + low WCC), amyloidosis

25
Q

RA: Investigation and Management

A

Inv - RF (IgM Ab), anti-CCP (can detect 10yrs before, more specific), all need XR hands and feet

-> start DMARD asap (methotrexate, monitor FBC/ LFTs), + bridging prednisolone, PO/ IM steroids for flares

*Use CRP and disease activity (DAS28 score) to assess response to treatment

26
Q

RA: Poor Prognosis

A

RF/ anti-CCP positive
Poor functional status at presentation
Early erosions on XR
HLA DR4
Insidious onset
Extra-articular disease

27
Q

RA vs OA

A

Rheumatoid

More women
Any age
MCP PIP (No DIP)
Sig morning stiffness
Bilateral
Systemic issues
XR - loss of joint space, juxta articular OP, swelling, later periarticular erosions and subluxation

28
Q

OA: Xray

A

L - Loss of joint space
O - Osteophytes
S - Subchondral sclerosis
S - Subchondral cysts

29
Q

Rotator Cuff Muscles

A

S - Supraspinatus - abducts arm
I - Infraspinatus - external rotation
T - teres minor - adducts and external rotation
S - Subscapularis - adducts and internal rotation

30
Q

Septic Arthritis

A

Joint inflammation 2nd to infection, ^knee

Cause - staph aureus, gonorrhea in young sexually active

= hot swollen red joint, v movement, fever

Inv - synovial fluid sampling, blood cultures

-> IV flucloxacillin 4-6wks

31
Q

Sjogren’s

A

AI disorder affecting the exocrine glands, causes dry mucosal surfaces

Cause - primary or 2nd to RA or CTD (>10yrs)

= 9F:1M, keratoconjunctivitis sicca, dry mouth, vaginal dryness, recurrent parotitis, Schirmer’s (filter paper)

Inv - RF, ANA (70%), anti-Ro/ anti-La

-> artificial tears and saliva, pilocarpine

Comp - lymphoid malignancy

32
Q

Still’s Disease in Adults

A

Idiopathic inflammatory condition

= 15-25yrs or 35-45yrs, arthralgia, salmon pink mp rash, fever (worse in afternoon, when others worse), nodes

Inv - ^ferritin, negative RF/ ANA, Yamaguchi criteria

-> NSAIDs, steroids after 1wk, methotrexate or anti-TNF if persists

Comp - macrophage activation syndrome (v ESR, = non-blanching rash, DIC, anaemia)

33
Q

Systemic Sclerosis

A

= 4F:1M, hardened sclerotic skin

Limited: ^distal limbs/ face, e.g., CREST, anti-centromere

Diffuse: ^proximal limbs/ trunk, + lung renal, anti-scl70

Scleroderma: tightening and fibrosis of skin, no organ

Inv - 90% ANA, 30% RF

-> if renal disease start ACEi

34
Q

Vitamin D Supplementation

A

> 65yrs
Housebound
Pregnant/ BF
6m - 5yrs (if <500ml formula/ day)

35
Q

Dactylitis

A

Inflammation of a digit

Cause - psoriatic/ reactive arthritis, sickle cell, TB, sarcoid, syphilis

36
Q

Marfan’s Syndrome

A

AD, CTD, defect in protein fibrillin 1 (FBN1, Chr15)

= tall, ^arm span, high-arched palate, pectus excavatum, pes planus, scoliosis, dilated aortic sinuses (AR/ dissection/ aneurysm), MV prolapse, pneumothorax, dural ectasia, upward lens dislocation

Inv - regular ECHO

37
Q

EDS

A

AD, CTD affecting type 3 collagen

= elastic fragile skin, hypermobility, dislocations, bruising

Comp - AR, MV prolapse, aortic dissection, SAH, angioid retinal streaks

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