Journal
AMERICAN JOURNAL OF KIDNEY DISEASES
Volume 42, Issue 6, Pages 1149-1153Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1053/j.ajkd.2003.08.015
Keywords
small-vessel vasculitis (SVV); diffuse alveolar hemorrhage (DAH); plasmapheresis; apheresis; antineutrophil cytoplasmic antibodies (ANCAs)
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Funding
- NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES [P01DK058335] Funding Source: NIH RePORTER
- NIDDK NIH HHS [5-P01-DK-58335-01-03] Funding Source: Medline
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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.
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