vasculitis review: intern conference jason haag. overview nomenclature of vasculitis overview of...
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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
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 VasculitisHenoch 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