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Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis.

BACKGROUND
Unlike Goodpasture's syndrome with diffuse alveolar hemorrhage (DAH), there are few studies examining therapy for patients with DAH associated with antineutrophil cytoplasmic antibody (ANCA)-associated small-vessel vasculitis (SVV).


METHODS
We performed a retrospective review of all such patients presenting to our institution between 1995 and 2001. All patients were treated with apheresis and induction immunosuppressive therapy; namely, intravenous methylprednisolone and/or intravenous cyclophosphamide.


RESULTS
DAH resolved with apheresis in 20 of 20 patients (100%) with 6.4 (average) treatments. There were no complications of therapy. Half the patients (7 of 14) who also presented with azotemia were discharged with improved renal function.


CONCLUSION
Patients with ANCA-related SVV and DAH benefit from prompt initiation of apheresis coupled with aggressive immunosuppressive therapy. Such therapy can be lifesaving with respect to the pulmonary component of this syndrome.