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