title: Treatment of antineutrophil cytoplasmic antibody-associated vasculitis.
abstract: OBJECTIVE
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Pulmonary Sciences and Critical Care Medicine, University of Colorado School of Medicine, Aurora, Colorado, USA.
authors.forename: Eric N
authors.initials: EN
authors.lastname: Rich
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Brown
mesh_terms: D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000175:diagnosis / Q000188:drug therapy*; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D019468:Disease Management; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects* / Q000627:therapeutic use*; D011379:Prognosis; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide