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Vasculitis Review: Intern Conference

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

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Vasculitis Review: Intern Conference

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  1. Vasculitis Review:Intern Conference Jason Haag

  2. Overview • Nomenclature of vasculitis • Overview of most important diseases • Quiz review

  3. 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)

  4. Jennette and Falk, Curr Op Rheumatol, 2007

  5. 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

  6. Medium Vessel Vasculitides (3) • Polyarteritis nodosa (PAN) • Buerger’s = Thromboangiitis obliterans • Kawasaki’s disease = Mucocutaneous LN syndrome

  7. 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

  8. 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

  9. 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

  10. Takayasu’s arteritis MRA

  11. 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

  12. 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

  13. Polyarteritis Nodosa (PAN)

  14. 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

  15. 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

  16. Medium Vessel Vasculitis:Kawasaki’s Disease

  17. 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

  18. 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

  19. Small Vessel Vasculitis: Goodpasture’s Syndrome • Hemoptysis/hematuria • Linear IgG deposits across basement membrane • Tx: • Stx, plasmapheresis

  20. 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= 48h T = 24h

  21. 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

  22. Small Vessel Vasculitis: Cryoglobulenemia • Nonspecific Sx • Arthralgias, myalgias, fever, glomerularneprhitis • Palpable pupura • Associated with • Hep C, RA, SLE • Low complement levels • Tx primary disease

  23. Quiz Review

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