(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