Immune Complex-Associated Small-Vessel Vasculitides

Immune Complex-Associated Small-Vessel Vasculitides

 • Henoch-Schönlein Purpura, Cryoglobulinemia


90% in children; ♂>♀; often after URI; in adults, more severe presentation, possible a/w malignancy

 • Sx: classic tetrad of 

    1) palpable purpura (100%, usually on LEs/buttocks =dependent areas)

    2) colicky abdominal pain (60%)

    3) arthritis (75%)

    4) renal involvement (40-50%, proteinuria, microscopic hematuria to RPGN)

 • Rx: children: supportive, usually self-limited; adults may require immunosuppression: steroids, dapsone. NSAIDs if mild GI/ arthralgias


Immunoglobulins that precipitate at low temperatures and re-dissolve on rewarming

 • Type 1: Monoclonal (usually IgM or IgG), associated with Waldenstrom’s, MM

     o Sx: peripheral neuropathy, renal impairment, hyperviscosity (Raynaud’s, digital ischemia, livedo), vasculitis

• Type 2: “Mixed” monoclonal IgM against polyclonal IgG (often IgM with RF activity), associated with HCV, HIV, HBV, EBV

 • Type 3: “Mixed” polyclonal Ig (IgM or IgG) against polyclonal Ig (IgM or IgG), associated with CTDs, lymphoproliferative disorders, HCV

     o Sx: palpable purpura, arthralgias, myalgias

 • Rx: treat underlying cause (e.g., HCV); prednisone ± 2nd immunosuppressive agent (RTX, CYC); consider plasma exchange for Type 1

#SmallVessel #Vasculitides #differential #diagnosis #rheumatology #management #vasculitis
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