title: Improvements in treatment strategies for patients with antineutrophil cytoplasmic antibody-associated rapidly progressive glomerulonephritis.
abstract: The course of rapidly progressive glomerulonephritis (RPGN) caused by antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is often life-threatening, especially in the elderly when pulmonary involvement and/or severely impaired renal function are present. Corticosteroids and cyclophosphamide are the first-line treatment, but ironically infection, not vascular events such as hemorrhage, caused by the vasculitis itself, is the most common cause of death of RPGN patients. Several new treatment strategies, such as leukocytapheresis (LCAP) and intravenous immunoglobulin (IVIg), have become available during the past decade and these treatments have made it possible to treat high-risk RPGN patients without inducing serious immunosuppressive states. In the present paper we review recent clinical trials of LCAP and IVIg therapy in patients with pauci-immune/ANCA-associated RPGN, and show improved clinical outcomes after using these new treatment strategies in our institution.
authors.affiliation: Department of Nephrology, Sendai Shakaihoken Hospital, Sendai, Japan. hottao@remus.dti.ne.jp
authors.forename: Osamu
authors.initials: O
authors.lastname: Hotta
authors.forename: Aki
authors.initials: A
authors.lastname: Ishida
authors.forename: Tomoyoshi
authors.initials: T
authors.lastname: Kimura
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Taguma
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D003520:Cyclophosphamide / Q000627:therapeutic use; D018450:Disease Progression; D005921:Glomerulonephritis / Q000276:immunology* / Q000628:therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007937:Leukapheresis; D010951:Plasma Exchange; D018805:Sepsis / Q000276:immunology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide