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Comprehensive Overview of Vasculitis: Nomenclature and Key Diseases

This review provides an in-depth overview of vasculitis, a group of diseases characterized by the inflammation of blood vessels, leading to vessel and tissue damage. It covers the nomenclature established by the Chapel Hill Consensus Conference of 1994 and classifies vasculitis into large, medium, and small vessel types. Notable conditions discussed include Giant Cell Arteritis, Takayasu's Arteritis, Polyarteritis Nodosa, and ANCA-associated vasculitis. The review includes a quiz to assess understanding of the nomenclature and key features of each vasculitis type, highlighting critical symptoms and treatment options.

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Comprehensive Overview of Vasculitis: Nomenclature and Key Diseases

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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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