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Small Vessel Vasculitides without Immune Complexes

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Topic updated on 11/08/16 2:24pm

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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Qbank (2 Questions)

TAG
(M1.CV.68) A 62-year-old Caucasian male presents to your office with hemoptysis and hematuria. On physical exam you note a saddle nose deformity. Laboratory results show an elevated level of cytoplasmic antineutrophil cytoplasmic antibody. Which of the following interventions is most appropriate for this patient? Topic Review Topic

1. Smoking cessation
2. IV immunoglobulin
3. Corticosteroids
4. Isoniazid
5. Discontinuation of ibuprofen

PREFERRED RESPONSE ▶
TAG
(M1.CV.80) A 21-year-old Caucasian male presents to your office with wheezing and rhinitis. Laboratory results show peripheral eosinophilia and antibodies against neutrophil myeloperoxidase. What is the most likely diagnosis? Topic Review Topic

1. Allergic bronchopulmonary aspergellosis
2. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
3. Cystic fibrosis
4. Paragoniums westermani infection
5. Pancoast tumor

PREFERRED RESPONSE ▶


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