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Vasculitis - Coggle Diagram
Vasculitis
Large Vessel
Giant Cell Arteritis
50+ yo
Dx: Bilateral temporal artery biopsy // angiography to follow
Rx: Steroids
Rx: Tocilizumab
PMR
Not really a vasculitis
Rx: PRednisone or methotrexate
Takayasu Arteritis
15-25 yo
Medium Vessel
PAN
Association with HBV
RX: Steroids / antivirals / plasmapheresis
Dx: focal segmental panmural necrotizing inflammation of medium sized vessel
Dx: Angiogram with aneurysm and stenosis of mesenteric or renal arteries, especially at branch points
Rx: Severe = steroids / cyclophosphamide
Rx: Mild = steroids / DMARDS
Primary angiitis of the CNS
Dx: Brain biopsy with granulomatous vasculitis
Rx: Steroids, cyclophosphamide
Maintenance with azathioprine or mycophenolate
Kawasaki
Rx: IVIG, aspirin
Rx multiple aneursyms: clopidogrel (IVIG also reduces aneurysms)
Giant aneurysm Rx: Warfarin prophylaxis
Small Vessel Vasculitis
Immune complex
Cryoglobulinemic vasculitis
Dx: Cryo, low C4 complement, positive RF
Rx: Antivirals
Rx: Severe or refractory = steroids + cyclophosphamide / plasmapharesis + rituximab
IgA Vasculitis
"Henoch Schonlein Purpura"
Arthralgia
Purpura
Abdominal Pain
Dx: Skin biopsy with leukocytoclastic vasculitis w/ heavy deposits of IgA and complement on immunofluorescence staining
Rx: Steroids and cyclophosphamide in adults
Hypersensitivity Vasculitis
Rx: Remove antigen, then if 1+ mo persistent symptoms = steroids or colchicine
ANCA Associated
Eosinophilic Granulomatosis with polyangitis
Kidney, GI, neuro involved: Cylophosphamide
Rx: Mild: Steroids
Dx: Clinical findings + eosinophilia + biopsy of fibrinoid necrosis with eosinophilic infiltration of vessell wall + extravascular granuloma formation
Asthma, nasal polyps, rhinitis
Churg Strauss
Microscopic Polyangiitis
Rx: Steroids + cyclophosphamide / rituximab
Maintenance with azathioprine, mycophenolate, rituximab
Prognosis worse in pulm hemorrhage or rapidly progressive glomerulonephritis
Bronchiectasis
Dx: Nongranulomatous pauci immune vasculitis
Granulomatosis with polyangiitis
Relapse Rx: Rituximab
Rx: nonsevere = high dose steroids + methotrexate / mycophenolate mofetil
Rx: Severe = high dose steroids + cyclophosphamide / rituximab
Maintenance with azathioprine, mycophenolate, rituximab
Dx: pauci immune necrotizing granulomatous vasculitis
Localized subtype: young F -- more prone to relapse, mainly ear, nose, throat involvement
Systemic form: anti PR3 positive
Most common ANCA associated vasculitis
"Wegeners"
c-ANCA