Treatment of antineutrophil cytoplasmic antibody-associated vasculitis.

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Treatment of antineutrophil cytoplasmic antibody-associated vasculitis.

Curr Opin Pulm Med. 2012 Sep;18(5):447-54

Authors: Rich EN, Brown KK


PURPOSE OF REVIEW: The primary idiopathic small-vessel vasculitis syndromes include granulomatosis with polyangiitis, Churg-Strauss syndrome, and microscopic polyangiitis. These disorders are commonly referred to as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides and prominently affect the pulmonary vasculature. Although significant progress has been made in the management of these disorders, they continue to carry substantial morbidity and mortality as a result of both the underlying vasculitis as well as complications of its immunosuppressive therapy. This review will focus on the recent advances in the management and longitudinal monitoring of ANCA-associated vasculitis.

RECENT FINDINGS: Cyclophosphamide and glucocorticoids are standard therapy, but carry measureable risk of treatment-related toxicity. The search for alternative therapies that are less toxic but similarly efficacious is continuing. Recent investigations suggest rituximab may be a well tolerated alternative to cyclophosphamide for the induction of remission, treatment of disease relapse, and as maintenance therapy.

SUMMARY: The ANCA-associated vasculitides are a group of disorders that commonly affect the pulmonary vasculature and represent a diagnostic and therapeutic challenge to the pulmonary clinician. Recent findings have expanded our ability to diagnose and treat these disorders with a focus on limiting treatment-related toxicity while inducing and maintaining remission.

PMID: 22854508 [PubMed - in process]

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