Rheum Flashcards
Temporal Arteritis
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
Polymyalgia Rheumatica
= >60yrs, morning stiffness in prox muscles, aching, no true weakness
Inv - ^ESR, normal CK, normal EMG
-> pred, reconsider if no drastic response
ANCA Vasculitis
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
Ankylosing Spondylitis
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
Behcet’s Syndrome
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
Bone Disorders: Labs
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
Chronic Fatigue Syndrome/ ME
> 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
Polymyositis
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
Dermatomyositis
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)
SLE
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
Discoid Lupus Erythematous
Benign AI disorder, <5% progress to SLE
= erythematous raised rash on head, may be scaly, photosensitive, scarring/ pigmentation
-> top steroid, hydroxychloroquine 2nd
Drug-Induced Lupus
= arthralgia, myalgia, malar rash, pleurisy (less renal/ nervous)
Cause - procainamide, hydralazine, isoniazid, phenytoin
Inv - ANA (100%), dsDNA negative, anti-histone Ab
Fibromyalgia
= F, 30-50yrs, widespread pain, tender points, lethargy, fibro fog
Inv - 9 pairs of tender points (likely if 11/18)
-> explain, aerobic exercise, CBT
Gout
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
Langerhans Cell Histiocytosis
Rare, abnormal proliferation of histiocytes
= child, bone pain, skull (punched out, beveled), proximal femur, cutaneous nodules, recurrent OM/ mastoiditis
McArdle’s Disease
AR, type V glycogen storage disease, v muscle glycogenolysis
= muscle pain and stiffness post-exercise, cramps
Inv - myoglobinuria, v lactate during exercise
Osteogenesis Imperfecta
AD, type 1 collagen disorder (brittle bone disease)
= child, fractures from minor trauma, blue sclera, deafness (otosclerosis), dental issues
Inv - normal bone profile
Osteomalacia
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
Polyarteritis Nodosa
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%)
Pseudogout
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
Psoriatic Arthrtis
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
Raynaud’s
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
Reactive Arthritis
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
RA - Features
= 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
RA: Investigation and Management
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
RA: Poor Prognosis
RF/ anti-CCP positive
Poor functional status at presentation
Early erosions on XR
HLA DR4
Insidious onset
Extra-articular disease
RA vs OA
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
OA: Xray
L - Loss of joint space
O - Osteophytes
S - Subchondral sclerosis
S - Subchondral cysts
Rotator Cuff Muscles
S - Supraspinatus - abducts arm
I - Infraspinatus - external rotation
T - teres minor - adducts and external rotation
S - Subscapularis - adducts and internal rotation
Septic Arthritis
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
Sjogren’s
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
Still’s Disease in Adults
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)
Systemic Sclerosis
= 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
Vitamin D Supplementation
> 65yrs
Housebound
Pregnant/ BF
6m - 5yrs (if <500ml formula/ day)
Dactylitis
Inflammation of a digit
Cause - psoriatic/ reactive arthritis, sickle cell, TB, sarcoid, syphilis
Marfan’s Syndrome
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
EDS
AD, CTD affecting type 3 collagen
= elastic fragile skin, hypermobility, dislocations, bruising
Comp - AR, MV prolapse, aortic dissection, SAH, angioid retinal streaks
