Antineutrophil cytoplasmic antibody-associated vasculitis complicated with diffuse alveolar hemorrhage: a study of 12 cases.

OBJECTIVE: To summarize the clinical features and therapeutic response of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis complicated with diffuse alveolar hemorrhage (DAH).

METHOD: A retrospective chart review of the patients having ANCA-associated vasculitis with DAH was made with regard to their clinical symptoms, laboratory test results, responses to therapy and outcomes.

RESULTS: During January 1994 to June 2007, 131 ANCA-associated vasculitis patients were admitted to Peking Union Medical College Hospital. During this period, 12 of these cases consisting of 9 males and 3 females with a mean age of 59.9 +/- 16.7 years developed DAH. The mean duration of vasculitis before the onset of DAH was 9.0 +/- 14.3 months (range: 0-48 months). Dyspnea was the most consistent presenting symptom, while frank hemoptysis occurred in only 5 cases at the onset of DAH. The most common extrapulmonary findings associated with DAH were glomerulonephritis (100%). Symptoms including fever and arthralgia-myalgia (91.7%) as well as complications involving gastrointestinal (41.7%), mucocutaneous (25%), ear-nose-throat (25%), cardiovascular (16.7%), and nervous system (16.7%) were also frequently seen. The Birmingham Vasculitis Activity Score at the onset of DAH was 21.8 +/- 4.9. All patients were treated with systemic corticosteroids combined with cyclophosphamide including methylprednisolone pulse therapy in 7 patients. In addition, plasmapheresis (41.7%), dialysis (25%), and mechanical ventilation (41.7%) were applied. The overall mortality rate was 58.3% (7 patients). Three patients died of fulminant DAH and respiratory failure during the first week of treatment. The other 4 patients died of septic shock (2 patients), severe heart failure (1 patient), and systemic fungus infection with septic shock (1 patient) 10 to 32 days after the onset of DAH.

CONCLUSION: DAH is the most serious complication of ANCA-associated vasculitis. The episode of DAH always occurs simultaneously with multiple system involvement. The most constant signs are newly developed dyspnea and new infiltration of bilateral lungs. Prompt bronchoalveolar lavage can be helpful for timely diagnosis of the patients without overt hemoptysis and a useful tool to exclude pulmonary infection.