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Catheter-based Endovascular Angioplasty for Fibrosing Mediastinitis-associated Pulmonary Vein Stenosis
Published on: August 26, 2025
Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis
Philip J Klemmer1, W Chalermskulrat, Michael S Reif
1Division of Nephrology and Hypertension, University of North Carolina, Chapel Hill 27599-7155, USA. philip_klemmer@med.unc.edu
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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