The antineutrophil cytoplasmic antibody-associated vasculitides.
Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome are small- to medium-vessel vasculitides linked by overlapping pathology and the presence of antineutrophil cytoplasmic antibodies (ANCA). Commonly referred to as the ANCA-associated vasculitides, these diseases are challenging to diagnose and to treat. Distinguishing the ANCA-associated vasculitides from other forms of vasculitis or nonvasculitic processes (such as infection) can be particularly difficult. This review describes the clinical and pathologic hallmarks of the ANCA-associated vasculitides, discusses the role of ANCA assays in diagnosis and treatment, and outlines an approach to the evaluation and management of these diseases.
Seo
Philip
P
Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Stone
John H
JH
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004423:Ear; D004802:Eosinophilia / Q000453:epidemiology; D005123:Eye; D006321:Heart; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney; D008168:Lung; D009666:Nose; D010525:Peripheral Nerves; D010614:Pharynx; D011379:Prognosis; D014657:Vasculitis / Q000188:drug therapy / Q000276:immunology* / Q000503:physiopathology*
D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide