Isolated Pulmonary Capillaritis

(aka Idiopathic Pauci-Immune Pulmonary Capillaritis)

Epidemiology

  • ANCA-negative variant has been reported to be the most frequent etiology of diffuse alveolar hemorrhage in one series
    [Diffuse Alveolar Hemorrhage with Underlying, Isolated Pauci-Immune Pulmonary Capillaritis. Am J Resp Crit Care Med 1997; 155: 1101-1109]

Physiology

  • Small vessel vasculitis (with capillaritis) confined to lung

Diagnosis

  • CBC: anemia
  • p-ANCA: may be positive or negative
  • Immunofluorescence: negative
  • ANA: negative
  • RF: negative
  • Complement: normal
  • OLB: may be necessary

Clinical

  • Diffuse Alveolar Hemorrhage (see [[Diffuse Alveolar Hemorrhage]])
  • Acute Respiratory Failure: may occur

Treatment

  • Corticosteroids + Cytoxan: typically good response
    • May recur

Prognosis

  • Death is infrequent with therapy

References

  • Diffuse Alveolar Hemorrhage with Underlying, Isolated Pauci-Immune Pulmonary Capillaritis. Am J Resp Crit Care Med 1997; 155: 1101-1109