Vasculitis Flashcards
Takayasu’s
Chronic inflammatory granulomatous aortitis
Asians, South Americans
Cause unclear
Clinical:
- young women <40
- limb claudication, dec brachial artery pulse, differential limb sBP > 20, subclavian arter or aortic bruit, abnormal angio
Inv
- angiogram - GOLD standard (shows beading, stenosis, aneurysms)
Mx:
- steroids +/- steroid-sparing immunosuppressant
- anti-IL-6R antagonist (tocilizumab)
- anti-CD 20 (rituximab)
- TNF antagonists
CGA
Giant cell arteritis
Clinical:
- constitutional symptoms, insiduous onset, jaw claudication, headache, scalp tenderness, PMR, aortic arch symptoms, visual disturbance
Inv:
- elevated ESR, CRP, other acute phase reactatns
- anaemic of chronic disease
- temporal artery Bx - GOLD standard (may be delayed put to 2/52, slip lesions
- US temporal arteries
Mx:
- steroids
- methotrexate as steroid-sparing agent
- low dose aspirin for inc CV and cerebrovascular event risk
- anti-IL6R antagonists (tocilizumab)
Polyarteritis nodosa
PAN
Systemic necrotising vasculitis of medium-sized muscular arteries
Associated Hep B in up to 1/3, HepC, HIV, hairy cell leukaemia
Clinical:
- consitutional symptoms, skin, joints kidney, hypertension, GIT, nerves, coronary arteries, orchitis (spares lung often)
- ANCA negative
Inv:
- biopsy
- angiogram
Mx:
- prednisolone + cyclophosphamide –> pred + aza maintenance
Granulomatosis with polyangiitis (GPA)/Wegener’s Granulomatosis
Clinical:
- constitutional symptoms, ENT symptoms, pulmonary (haemorrhage), renal (rapidly progressive pauci-immune crescentic GN), skin, opthal, neuro
Inv:
- CNA+
- PR3+
- Bx: necrotising granulomatous inflammation
Mx:
- LIMITED: pred + aza/methotrexate
- severe systemic pred + cyclophosphamide
Churg-Strauss Syndrome
CSS = eosinophilic granulomatosis with polyangiitis
Eosinophilic-rich necrotising granulomatous inflammation of small vessels
ACR criteria: asthma, eosinophilia, mononeuropathhy, polyneuropathy, pulm infiltrates, paranasal sinus abnormalities, eosinophilic granulomas
Inv:
- ANCA + (usually pANCA in 30-40%)
Phenotypes:
Vasculitic ANCA +, renal disease
Eosinophilic ANCA -, cardiomyopathy
Mx:
- pred + cyclo
- mepolizumab (anti-IL5) if refractory
Anti-GBM disease
Pathogenic AutoAb against NC alpha 3 chain of Type IV collagen
Pulmonary-renal syndrome, often no prodrome
Rapidly progressive, crescentic GN
60-70% pulmonary involvement (Goodpasture’s)
Inv:
- anti-CBM Ab in serum
- 1/3 assoc with ANCA (often pANCA, MPO+)
- renal Bx diagnostic of renal involvement (linear IgG, C3 staining of capillary loops of glomeruli)
Mx:
- pred, cyclophosphamide, plasma exchange
Better prognosis = <30% crescents, creat < 300, not dialysis dependent at diagnosis
Henoch-schonlein purpura
Most common childhood vasculitis <5 years
Clinical:
- palpable purpura, arthritis, abdo pain, GIT bleeding, nephrits
- infection prodrome (beta-haemolytic strep)
- may be associated with solid-organ malignancies (adult)
Inv:
- skin Bx: IgA and C3 deposits
Mx:
- generally self-limited 4 weeks (91%)
- corticosteroid may help arthralgia and abdo pain
Prognosis dept on degree of renal involvement
No Rct on benefit of steroids in preventing nephritis or altering course of nephritis
Cryoglobulinaemic vasculitis
Cryoglobulin precipitates in the cold (4C) after 5-7 days, dissolves with rewarming
Clinical:
- purpura, arthralgia, weakness, neuropathy, membranoproliferative GN
Brouet classification:
TYPE I: monoclonal Ig (B cell lymphoproliferative disrders)
TYPE II: polyclonal IgG with monoclonal IgM assoc with RF activity (Hep C)
TYPE III: polyclonal IgG and IgM with RF activity (HepC, autoimmune diseases)
Inv:
- monoclonal gammopathy (particularly IgM)
- unexplained low C4
- unexplained high RF
- pseudothrombocytosis
- formation of erythrocyte rouleaux
Histopath:
- leukocytoclastic vasculitis (adults)
- membranoproliferative GN
- hyaline thrombi
- endoneural vasculitis
- unclassified systemic necrotising vasculitis
Mx:
- according to underlying disease
Poor prognostic marker: pulmonary and GIT involvement, GFR<60, age>65
