ANCA Glomerulonephritis and Vasculitis

Clinical journal of the American Society of Nephrology: CJASN

J. Charles Jennette¹ 

[1] Department of Pathology and Laboratory Medicine, Department of Medicine, and Kidney Center, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina..Department of Pathology and Laboratory Medicine, Department of Medicine, and Kidney Center, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina..

关键词: ANCA;    vasculitis;    Myeloblastin;    Antibodies;    Antineutrophil Cytoplasmic;    Granulomatosis with Polyangiitis;    Peroxidase;    Neutrophils;    Autoantibodies;    Autoimmunity;    Prevalence;    Microscopic Polyangiitis;    Serogroup;    Churg-Strauss Syndrome;    glomerulonephritis;    Neutrophil Activation;    Models;    Animal;    Recurrence;   

DOI  :  10.2215/CJN.02500317

学科分类：泌尿医学

来源: American Society of Nephrology

【 摘 要 】

ANCA vasculitis has an associated autoimmune response that produces ANCAs that induce distinct pathologic lesions. Pauci-immune necrotizing and crescentic GN is a frequent component of ANCA vasculitis. ANCA vasculitis is associated with ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). A diagnosis of ANCA vasculitis should always specify the serotype as MPO-ANCA positive, PR3-ANCA positive, or ANCA-negative. To fully characterize a patient, the serotype also should be accompanied by the clinicopathologic variant if this can be determined: microscopic polyangiitis, granulomatosis with polyangiitis (Wegener), eosinophilic granulomatosis with polyangiitis (Churg–Strauss), or renal-limited vasculitis. ANCA vasculitis is most prevalent in individuals >50 years old. There are racial/ethnic and geographic influences on the prevalence, serotype frequencies, and clinicopathologic phenotypes. There is clinical, in vitro, and animal model evidence that ANCAs cause disease by activating neutrophils to attack small vessels. Immunomodulatory and immunosuppressive therapies are used to induce remission, maintain remission, and treat relapses. Over recent years, there have been major advances in optimizing treatment by minimizing toxic therapy and utilizing more targeted therapy.

【 授权许可】

CC BY