Vasculitis Review: Intern Conference Jason Haag
Overview Nomenclature of vasculitis Overview of most important diseases Quiz review
Nomenclature Vasculitis: Inflammation on blood vessels causing vessel and tissue damage Classification is according to the Chapel Hill Consensus Conference of 1994 Large-vessel vasculitides (2) Medium-vessel vasculitides (3) Small-vessel vasculitides (many) ANCA-associated (5) Non-ANCA-associated (many)
Jennette and Falk, Curr Op Rheumatol, 2007
Large Vessel Vasculitides (2) Giant cell arteritis (formerly called temporal arteritis) Takayasu’s arteritis Distinguished only by age of pt > or < 50 yo Both involve the aorta and its primary branches
Medium Vessel Vasculitides (3) Polyarteritis nodosa (PAN) Buerger’s = Thromboangiitis obliterans Kawasaki’s disease = Mucocutaneous LN syndrome
Small Vessel Vasculitides (many) ANCA-associated (5) Wegener’s granulomatosis (WG) Churg-Strauss syndrome (CSS) Microscopic polyangiitis (MPA) – no granulomas Drug-induced ANCA vasculitis Idiopathic
Small Vessel Vasculitides (many) Non-ANCA-associated (many) Infectious (Hep B, Hep C, HIV, Parvo, CMV, EBV) CVD-related (RA, SLE, Bechet’s, Sjogren’s) Drug-related (cutaneous leukocytoclastic vasculitis) Henoch-Schonlein Purpura (HSP) – IgA deposits Goodpasture’s disease Cryoglobulinemia IBD-related Urticarial vasculitis Paraneoplastic
Large Vessel Vasculitis: Takayasu’s arteritis Affects women < 40 yo Prepulseless phase: systemic sx Pulseless phase: arm claudication, HA, dizziness, visual changes, decreased arm pulses or pressure differential, subclavian/abdominal bruits Tx with Prednisone Fever, weight loss, fatigue
Takayasu’s arteritis MRA
Large Vessel Vasculitis: Giant Cell Arteritis Sx: fatigue, HA, visual changes, scalp tenderness, jaw claudication Often associated with PMR (pain and stiffness in neck, shoulder, pelvic girdles) Prompt corticosteroids prevent blindness
Medium Vessel Vasculitis: Polyarteritis Nodosa (PAN) Peaks in 5th decade, slight male preponderance Sx: constitutional sx, skin lesions (purpura, livedo reticularis, ulcers, nodules), mononeuritis multiplex, hematuria, abdominal pain, testicular pain Aneurysms and narrowing on MRA Associated with Hep B Tx with Prednisone, cyclophosphamide
Polyarteritis Nodosa (PAN)
Medium Vessel Vasculitis: Buerger’s Disease Young, 20-40 y.o Male Inflammation/thrombosis in hands/feet pain Recent or current tobacco use Tx with smoking cessation
Medium Vessel Vasculitis: Kawasaki’s Disease Classic Dx 5 days of fever + 4 of 5 criteria Erythema/cracking of lips strawberry tongue Trunkal rash Swelling of hands/feet Conjuctivitis Cervical LAD Also can have joint pain, coronary aneurysms, myocarditis Tx: IVIG
Medium Vessel Vasculitis: Kawasaki’s Disease
Small Vessel Vasculitis: Churg-Strauss Syndrome Middle-aged adults Asthma, eosinophilia (>10%), neuropathy (mono- or poly- in stocking/glove), pulmonary infiltrates ANCA+ vasculitis Tx: Prednisone, cyclophosphamide
Small Vessel Vasculitis: Wegener’s Granulomatosis Granulomas in upper resp tract (sinusitis, otitis, epistaxis), lower resp tract (hemoptysis), and kidneys (GN), can involve eye and nose Tx: Stx, cyclophosphamide
Small Vessel Vasculitis: Goodpasture’s Syndrome Hemoptysis/hematuria Linear IgG deposits across basement membrane Tx: Stx, plasmapheresis
Small Vessel Vasculitis: Bechet’s Syndrome Highest in Iran, Turkey, and Japan Recurrent oral and genital ulcers Uveitis causes blindness in 10% Skin pathergy > 2mm nodule after 48 hours Multiple systems involve Venous thrombosis Tx: Stx, TNF blocker T= 0 T = 24h T= 48h
Small Vessel Vasculitis Henoch Schonlein Purpura Most common in children aged 3-15, but also occurs in adults Presents with classic tetrad of purpuric rash, abdominal pain, renal disease, and arthralgias Can progress to renal failure (adults) Tx: Pain control, Stx if neccessary
Small Vessel Vasculitis: Cryoglobulenemia Nonspecific Sx Arthralgias, myalgias, fever, glomerularneprhitis Palpable pupura Associated with Hep C, RA, SLE Low complement levels Tx primary disease
Quiz Review