Small Vessel Vasculitis

Aka: Small Vessel Vasculitis, Vasculitis of Small Vessels, ANCA-Associated Vasculitis, Antineutrophil Cytoplasmic Antibody Vasculitis

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II. Epidemiology: Most common Small Vessel Vasculitis

  1. Older Adults: ANCA-associated Small Vessel Vasculitis
  2. Children: Henoch-Schonlein Purpura

III. Type: ANCA-associated small-vessel Vasculitis

  1. Non-Granulomatous Disease
    1. Microscopic Polyangiitis
      1. Most common ANCA Vasculitis
      2. Lung and renal findings
    2. Drug-Induced Vasculitis (7-21 days after drug onset)
      1. Penicillin and Aminopenicillins
      2. Sulfonamides
      3. Allopurinol
      4. Thiazides
      5. Quinolones
      6. Hydantoins (e.g. Phenytoin)
      7. NSAIDs
      8. Propylthiouracil
      9. Hydralazine
  2. Granulomatous Disease
    1. Churg-Strauss Syndrome
      1. Associated with Asthma and Eosinophilia
    2. Granulomatosis with Polyangiitis (previously known as Wegener's Granulomatosis)
      1. Lung, Renal, and upper respiratory signs

IV. Type: non-ANCA associated Small Vessel Vasculitis

  1. Paraneoplastic Small Vessel Vasculitis
  2. Inflammatory Bowel Disease
  3. Immune Complex Small Vessel Vasculitis
    1. Henoch-Schonlein Purpura (Lung and skin signs)
    2. Cryoglobulinemia (Lung and skin signs)
    3. Systemic Lupus ErythematosusVasculitis
    4. Rheumatoid Arthritis
    5. Goodpasture's Syndrome
    6. Sjogren's Syndrome
    7. Drug-induced immune-complex Vasculitis
    8. Infection-induced immune-complex Vasculitis
    9. Behcet's Disease

V. Symptoms

  1. Fever
  2. Weight loss
  3. Malaise
  4. Myalgias and Arthralgias
  5. Dyspnea
  6. Cough (Hemoptysis may be present)
  7. Diarrhea
  8. Nausea or Vomiting
  9. Abdominal Pain

VI. Signs

  1. Dermatologic findings (Cutaneous Small Vessel Vasculitis)
    1. Palpable Purpura typically on the lower legs (duration longer than 24 hours)
    2. Urticaria
  2. Pulmonary findings
    1. Interstitial Lung Disease
    2. Pulmonary Hemorrhage
  3. Neurologic findings
    1. Peripheral Neuropathy
  4. Gastrointestinal findings
    1. Fecal blood positive

VII. Differential Diagnosis

  1. Embolic disease
  2. Sepsis
  3. Lymphoma
  4. Leukemia
  5. Myelodysplastic condition

VIII. Labs

  1. Antineutrophil Cytoplasmic Antibodies (ANCA)
  2. Complete Blood Count (CBC)
    1. Normocytic Anemia
    2. Thrombocytosis
  3. Chemistry profile (e.g. Chem8)
    1. Renal Function tests may show Renal Insufficiency
  4. Liver Function Tests
    1. Increased liver enzymes
  5. Fecal Occult Blood
  6. Urinalysis (Glomerulonephritis)
    1. Hematuria
    2. Proteinuria

IX. Radiology: Chest XRay

  1. Interstitial Lung Disease findings may be seen

X. Diagnostics

  1. Biopsy from symptomatic site (e.g. skin, lung, Kidney)

XI. References

  1. Calabrese in Ruddy (2001) Kelley's Rheum, p. 1165-7
  2. Mansi (2002) Am Fam Physician 65(8):1615-20 [PubMed]

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