title: [Anti-neutrophil cytoplasmic antibody-associated vasculitis. Clinical aspects and treatment].
abstract: Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis, comprise a group of diseases characterized by inflammation of the wall of small vessels. We analyzed epidemiological and clinical characteristics in a series of 47 patients, 23 (49%) with Wegener granulomatosis (WG), 15 (32%) with microscopic polyangiitis (MPA) and nine (19%) with renal limited vasculitis (RLV). The mean age at onset of symptoms was 50.7 ± 14.9 years. The most frequent clinical manifestation was renal involvement in 41 (87%), followed by pulmonary manifestations in 26 (55%) and ENT involvement in 17 (36%). In 26 (55%) it presented with simultaneous pulmonary and renal involvement. The most frequent clinical category was the generalized form in 23 (49%), followed by the severe form in 18 (38%). Eighty nine percent of patients had positive ANCA test. Four (8%) received no immunosuppressive treatment. Of the 43 patients who were treated, 29 (67%) achieved complete remission with an average length of remission of 35.3 months. Eleven (26%) had a relapse, ten (91%) had a major relapse and one had a minor relapse. Twelve (28%) patients died, seven died early and five late during the course of the disease. Fifteen (31%) progressed to chronic renal failure. All 26 patients in follow-up had response to treatment and 20 (77%) were in remission at the end of the study. Despite the improvements achieved with immunosuppressive treatments, morbidity and mortality rates in ANCA-associated vasculitis remain high.
authors.affiliation: Unidad de Inmunología e Histocompatibilidad, Hospital Dr. Carlos G. Durand, Buenos Aires, Argentina. virpaolini@gmail.com
authors.forename: María Virginia
authors.initials: MV
authors.lastname: Paolini
authors.forename: Juan Pablo
authors.initials: JP
authors.lastname: Ruffino
authors.forename: Diego S
authors.initials: DS
authors.lastname: Fernández Romero
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000188:drug therapy / Q000276:immunology* / Q000401:mortality; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis*; D001118:Argentina / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000098:blood supply; D007674:Kidney Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D008297:Male; D055953:Microscopic Polyangiitis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D008875:Middle Aged; D012074:Remission Induction; D013997:Time Factors; D055815:Young Adult
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007166:Immunosuppressive Agents
keywords: ANCA-associated vasculitis; Wegener granulomatosis; microscopic polyangiitis; renal limited vasculitis