Rheumatology Flashcards

Question

Raynaud's phenomenon

Answer 1

**cause:** primary or secondary (ScL, SLE or other connective tissue disease) **clinical:** colour change with blanching cyanosis and hyperanaemia **diagnosis:** - symmetric episodic attacks - no evidence PVD - no gangrene/digital pitting/tissue injury - negative nailfold capillary examination - negative ANA and normal ESR

Answer 2

**pathogenesis:** auto Ab against type IV collagen **clinical:** pulmonary-renal syndrome w/o prodrome - renal: rapidly progressive GN (20% cresenteric) - pulmonary (70%): Goodpasture's syndrome **diagnosis:** - 1/3 pANCA MPO+ - anti-GBM Ab - renal biopsy: IgG, C3 staining in capillary loops glomeruli **treatment:** prednisone, cyclophosphamide

Answer 3

**definition:** multisystem granulomatous disorder of unknown etiology causing noncaseating granulomas in multiple organs **clinical:** - _LN (40%)_: bilateral hilar adenopathy, swelling parotid, salivary - _pulmonary:_ reticular opacities - _skin (25%)_: papular, nodular, plaque lesions, erythema nodosum - _ocular (25%):_ uveitis, keratoconjunctivitis sicca - _musculoskeletal (10%):_ polyarthritis, myositis - _other:_ endocrine, renal, CNS, CVS (arrythmia, HB), GI **symptoms:** cough, dyspnoea, chest pain, fatigue, malaise, weight loss **lofgren sx:** EN, hilar LN, migratory polyarthritis, fever **diagnosis:** - _AI_: ANA +ve 90%, RF, anti-Ro/anti-La, ACE (monitor) - _serum_: increased eosinophils, anaemia/thrombocytopaenia/leukopaenia, elevated ACE (75%), hypergammaglobulinaemia - _biopsy_: noncaseating granuloma lung

Answer 4

**incidence:** RARE in childhood **pathophysiology:** idiopathic systemic necrotizing vasculitis affecting small/medium muscular arteries of every system except lungs - may be secondary to Hep B/Hep C/hairy cell leukaemia **clinical:** mean age 9 years - _systemic:_ fever, fatigue, myalgia - _skin:_ purpura, livedo reticularis, bullous/vesicular eruptions, tender subcut nodules - _renal:_ afferent arteriolar narrowing with ischaemia, HTN - _neuro_: mono/polyneuropathy - _GI:_ abdominal pain/ischaemia (small\>large bowel, weight loss - _msk_: myaglia (elevated CK) - _CVS_: ischaemia, infarction **infantile form:** \<2yrs, coronary arteries with aneurysm/CCF **diagnostic cx:** biopsy arteries + skin/muscle/HTN/PN/renal sx **treatment:** steroids, MTX, cyclophosphamide **prognosis:** 1 yr survival 50%, 5 yr survival 13%

Answer 5

**incidence:** most common childhood vasculitis **clinical:** mean 6-7yrs - _skin_ (100%): purpura, petechiae - _GI_: abdo pain (30%), nausea, vomiting, ileal-ileal intussusception (3.5%), ischaemia - _arthritis_ (40%): migratory, oligoarticular, LL - _renal_ (50%): IgA nephropathy (haematuria, casts, proteinuria) within 6 months with secondary HTN - other: headaches, seizures, ICH, keratitis, uveitis **diagnosis:** skin changes + 1 other sx **management:** NSAIDs, steroids, immunosuppresants if renal **prognosis:** 1/3 reoccur within 4 months

Answer 6

**incidence:** boys\>girls \<5yrs asian **pathophysiology:** unknown etiology possibly infective - seasonal with clusters - familial clustering - fever due to cytokines TNF/IL-6 and minimally responsive to anti-pyretics **clinical:** fever \>5 days with 4 of 5 of the following **1.** polymorphous rash (70-90%) **2.** change of extremities (50-80%): oedema/erythema palms/soles, periungal desquamation wk 2/3, arthritis **3.** cervical LN: 1 at least \>1.5cm (25-70%) **4.** bilateral non-purulent conjunctivitis (\>90%), anterior uveitis **5.** mucositis (90%): cracked lips, strawberry tongue - other: pneumonitis, URTI, D/V, sterile pyuria **complications:** - _cardiac_: coronary artery aneurysm/arteritis (\<1yr or \>9yr, male, fever\>14d), pericardial effusion, valvular regurg - CVS: peripheral arterial obstruction - other: SNHL, macrophage activation syndrome, behavioural issues **diagnosis:** - TTE at 2 weeks/8 weeks - serum: elevated ESR/CRP/WCC/platelets/transaminases - CSF: pleiocytosis **management:** IVIG 2g/kg within 10 days, high dose aspirin 30mg/kg **prognosis:** mortality 0.1%, coronary aneurysm (1% treated, 30% untreated)

Answer 7

**incidence:** most common idiopathic inflammatory myopathy of childhood, F\>M **clinical:** 5-10yrs symmetrical proximal muscle weakness: diffculty stairs, Gower's sign, swallowing issues _PLUS 2 of the following cutaneous sx:_ 1. heliotrope dermatitis: purple rash on eyelids with periorbital oedema 2. Gottran's papules: red papules/plaques to knuckles other: nail-fold capillary changes, skin ulcers, calcinosis skin/muscle, non-erosive arthritis, lisodystrophy, anorexia, fever, cardiac disease, ILD **diagnosis:** - _AI:_ positive polymyositis (Pm) or scleroderma (Scl) antibodies, ANA (80%) - _serum_: elevated CK, anaemia, lymphopaenia - _EMG:_ myopathy **treatment:** steroids, MTX, IVIg, cyclosporine **complications:** OP, calcinosis (delayed tx)

Answer 8

DAMP AS RHINO (4 of 11): **D**iscoid rash **A**NA + **M**alar rash **P**hotosensitivity **A**rthritis **S**erositis (lung/heart) **R**enal involvement: \>0.5g protein/casts **H**aem abnormality: anaemia, thrombocytopaenia, cytopaenia **I**mmune abnormalities: dsDNA, Sm, LAC, aCL **N**euro abnormalities: seizures, psychosis **O**ral/nasal ulcers

Answer 9

**definition:** idiopathic relapsing febrile non suppurative panniculitis in 30-60yo females usually affecting the lower limbs

Answer 10

**incidence:** 8% SJIA **definition:** secondary form HLH **clinical:** fever, rash, HSM **diagnosis:** - ferritin\>684 PLUS - platelets\<181 - AST\>48 - TG\>156 - Fibrinogen\<360 **prognosis:** 30% mortality