DIFFUSE ALVEOLAR HEMORRHAGE WITH UNDERLYING ISOLATED, PAUCIIMMUNE PULMONARY CAPILLARITIS

Diffuse alveolar hemorrhage (DAH) resulting from pulmonary capillariti s typically accompanies the systemic vasculitides and collagen vascula r diseases. Isolated pulmonary capillaritis and DAH without systemic d isease occurs in patients with antineutrophil cytoplasmic antibodies. However, isolated pulmonary capillaritis and DAH is not described for patients without clinical or serologic evidence for an underlying syst emic disease. To describe such patients, we undertook a retrospective chart review of 29 patients with DAH and biopsy-proven pulmonary capil laritis from seven Denver hospitals. Eight (28%) were diagnosed with i solated pulmonary capillaritis without clinical, serologic, or histolo gic evidence of an associated illness. Their median age was 30 yr. No association with occupational or drug exposures was identified. All ha d lower respiratory tract symptoms; seven had upper respiratory tract symptoms. None demonstrated systemic disease or evidence of glomerulon ephritis. All were antineutrophil cytoplasmic antibody negative. Other serologies were not significant where measured. Direct immunofluoresc ence studies of lung tissue were negative in five. Six presented with acute respiratory failure, four requiring mechanical ventilation. One died during initial hospitalization; seven survived. Median follow-up is 43 mo (7 to 73 mo). Five remain in remission. Two experienced multi ple recurrences of DAH but without development of systemic disease whi le on therapy. Herein we characterize DAH and isolated pulmonary capil laritis in the absence of clinical, serologic, or histologic evidence indicating an accompanying systemic illness. The prognosis for this gr oup appears favorable.