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Introduction
  • Characteristics of small vessel vasculitides WITHOUT immune complexes
    • lack of immune complex deposition
    • affect the respiratory system
    • have strong relationship with ANCA antibodies
  • Examples include
    • eosinophilic granulomatosis with polyangitis
      • p-ANCA
      • formally known as Churg-Strauss syndrome
    • granulomatosis with polyangiitis (GPA)
      • c-ANCA
      • formally known as Wegener's granulomatosis
    • microscopic polyangiitis (MPA)
      • p-ANCA
    • primary pauci-immune crescentic glomerulonephritis
      • p-ANCA
      • also known as renal-limited vasculitis
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)
  •  Overview
    • vasculitis affecting skin, heart, lung, and vessels
    • p-ANCA antibodies more common than c-ANCA
      • also known as antibodies against neutrophil myeloperoxidase
  • Presentation
    • respiratory symptoms
      • asthma and rhinitis
    • eosinophilia
  • Epidemiology
    • no age specifics
Granulomatosis with Polyangiitis (Wegener's Granulomatosis)
  • Overview
    • triad of necrotizing inflammation
      • focal necrotizing vasculitis
      • necrotizing granulomas of lung and upper respiratory tract
      • necrotizing glomerulonephritis
    • c-ANCA antibodies are a strong marker of disease
  • Presentation
    • respiratory symptoms
      • hemoptysis
      • perforation of nasal septum
      • saddle nose deformity
      • chronic sinusitis
      • cough
      • dyspnea
      • pneumonia/nodular densities on CXR
    • renal symptoms
      • crescentic glomerulonephritis
      • hematuria/red cell casts
    • other symptoms
      • otitis media
      • mastoiditis
  • Treatment
    • cyclophosphamide
    • rituximab
    • corticosteroids   
Microscopic Polyangiitis
  • Overview
    • vasculitis affecting skin, lung, GI, kidneys, brain
      • lesions of same age
    • p-ANCA antibodies are strong marker of disease
      • also known as antibodies against neutrophil myeloperoxidase 
  • Presentation
    • palpable purpura
    • glomerulonephritis
  • Epidemiology
    • all ages effected
    • reaction to drugs, infection, or immune disease
Primary Pauci-Immune Crescentic Glomerulonephritis
  • Vasculitis limited to kidney
  • "Paucity" of antibodies
  • Histopathology indistinguishable from renal involvement of microscopic polyangiitis or granulomatosis with polyangiitis
    • sometimes, may be mistakenly diagnosed with primary pauci-immune crescentic glomerulonephritis, then later be found to actually have MPA or GPA
 

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