Updated: 4/18/2020

Small Vessel Vasculitides without Immune Complexes

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Snapshot
  • A 34-year-old man presents to his doctor’s office for a rash of several weeks. He reports also having allergic rhinitis in the past few weeks, which was strange given that he normally has allergic rhinitis in the spring. Additionally, he reports having had childhood asthma, but in the past few months, he has had to use his inhaler for episodes of asthma. On physical exam, there is palpable purpura and urticarial plaques on the lower extremities and several skin nodules. Wheezes are heard on lung exam. Additional laboratory tests reveal peripheral eosinophilia with elevated IgE levels. (Eosinophilic granulomatosis with polyangiitis)
Introduction
  • Clinical definition
    • vasculitis mediated by neutrophils, rather than immune complexes
      • referred to as “pauci-immune” vasculitides
  • Pathogenesis
    • neutrophils directly damage vessel walls
      • ANCA antigens, proteinase 3 and myeloperoxidase, are proteins inside neutrophils that become expressed on the cell surface when activated
      • ANCA antibodies attach to these antigens and activate neutrophils, leading to vessel wall damage
    • affects small and medium-sized vessels of the skin and organs
    • cytoplasmic ANCA (c-ANCA) are directed against antigen proteinase 3 (PR3)
    • perinuclear ANCA (p-ANCA) are directed against antigen myeloperoxidase (MPO)
  • Studies
    • a biopsy may be needed to establish a diagnosis
    • direct immunofluorescence of skin biopsy is usually negative
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)
  • Clinical definition
    • necrotizing granulomatous vasculitis with eosinophilia affecting multiple organs
  • Presentation
    • skin involvement
      • palpable purpura
      • urticaria
      • skin nodules
    • upper respiratory involvement
      • chronic sinusitis
      • allergic rhinitis
      • nasal polyps
    • lung involvement
      • asthma
    • neurologic involvement
      • peripheral neuropathy
        • foot or wrist drop
    • can also affect the heart, gastrointestinal system, and kidneys
  • Studies
    • histology
      • necrotizing vasculitis
      • granulomas
      • eosinophilia
    • laboratory studies
      • peripheral eosinophilia
      • ↑ IgE
      • + MPO-ANCA/p-ANCA (anti-myeloperoxidase)
  • Treatment
    • systemic glucocorticoids
    • cyclophosphamide for severe, multi-organ disease
Granulomatosis with Polyangiitis (Wegener Granulomatosis)
  • Clinical definition
    • necrotizing granulomatous c-ANCA-associated vasculitis affecting the upper respiratory tract, lungs, kidneys, and skin
  • Presentation
    • skin and mucosal involvement
      • palpable purpura and ulcers
      • friable, red gingivae
        • "strawberry gums"
      • oral ulcers
    • upper respiratory tract involvement
      • nasal ulcers
      • perforation of the nasal septum
      • chronic sinusitis, otitis media, or mastoiditis
    • lung involvement
      • hemoptysis
      • cough
      • dyspnea
    • renal involvement
      • hematuria
  • Studies and imaging
    • urine studies
      • hematuria
      • red cell casts
    • histology
      • focal necrotizing vasculitis
      • necrotizing granulomas in the respiratory tract
      • necrotizing glomerulonephritis
    • laboratory studies 
      • + PR3-ANCA/c-ANCA (anti-proteinase 3)
    • chest radiography
      • large nodular densities
  • Treatment
    • cyclophosphamide
    • corticosteroids
    • mepolizumab, a humanized monoclonal antibody, approved by FDA in 2017
Microscopic Polyangiitis
  • Clinical definition
    • necrotizing p-ANCA-associated vasculitis affecting the lungs, kidneys, and skin
  • Presentation
    • skin involvement
      • palpable purpura
      • ulcers
    • no upper respiratory involvement (in contrast to granulomatosis with polyangiitis)
    • pulmonary involvement
      • hemoptysis
      • dyspnea
    • renal involvement
      • hematuria
  • Studies
    • urine studies
      • hematuria
      • red cell casts
    • histology
      • necrotizing vasculitis
      • no granulomas
    • laboratory studies
      • + MPO-ANCA/p-ANCA (anti-myeloperoxidase)
  • Treatment
    • cyclophosphamide
    • corticosteroids

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Questions (4)
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(M1.CV.13.68) 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? Tested Concept

QID: 101022
1

Smoking cessation

4%

(2/46)

2

IV immunoglobulin

26%

(12/46)

3

Corticosteroids

52%

(24/46)

4

Isoniazid

4%

(2/46)

5

Discontinuation of ibuprofen

4%

(2/46)

M 3 E

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(M1.CV.12.80) 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? Tested Concept

QID: 100596
1

Allergic bronchopulmonary aspergellosis

4%

(1/25)

2

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

92%

(23/25)

3

Cystic fibrosis

4%

(1/25)

4

Paragoniums westermani infection

0%

(0/25)

5

Pancoast tumor

0%

(0/25)

M 2 D

Select Answer to see Preferred Response

Evidence (3)
EXPERT COMMENTS (8)
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