Respiratory failure in ANCA-associated vasculitis.
Respiratory failure in ANCA-associated vasculitis.
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DOI：
10.1378/chest.110.2.357
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发表时间：
1996-08
期刊：
影响因子：
9.6
通讯作者：
J. T. ter Maaten;C. Franssen;R. Gans;Robert J.M. Strack Van Schijndel;S. Hoorntje
中科院分区：
文献类型：
--
作者：
J. T. ter Maaten;C. Franssen;R. Gans;Robert J.M. Strack Van Schijndel;S. Hoorntje
目的评估1985年1月至1993年1月期间在我院门诊抗神经细胞胞质自身抗体（ANCA）检测阳性的所有患者中呼吸衰竭的患病率、临床表现和病程。设计病例系列分析。设置在荷兰的三所教学医院。患者220例疑似血管炎和/或肾小球肾炎的患者，通过间接免疫荧光和酶联免疫吸附试验检测ANCA阳性。结果62例患者有肺部受累。9例发生急性呼吸衰竭。其中2例呼吸衰竭与感染有关，7例与ANCA相关性血管炎有关。这7例患者均表现为肺出血和弥漫性肺浸润。系统性血管炎的诊断得到了支持的肺-肾综合征的存在下，在所有患者，但一名患者的蛋白酶3或髓过氧化物酶抗原的抗体检测。抗肾小球基底膜抗体不存在。由于缺氧性呼吸衰竭、肺部双重感染和伴随的肾衰竭，死亡率很高。结论：ANCA相关肺部疾病患者中，每9例中就有1例发生血管炎所致急性呼吸衰竭。患者通常表现为肺浸润和咯血。血管炎的诊断可通过尿沉渣分析和ANCA靶抗原测定得到进一步支持。ANCA的早期发现对预后有积极影响，这一点还有待证实。
OBJECTIVE To assess the prevalence, clinical manifestations, and course of respiratory failure in all patients who tested positive for antineutrophil cytoplasmic autoantibodies (ANCA) in our clinics in the period between January 1985 and January 1993. DESIGN Case-series analysis. SETTING Three teaching hospitals in the Netherlands. PATIENTS Two hundred twenty consecutive patients suspected of having vasculitis and/or glomerulonephritis who tested positive for ANCA by indirect immunofluorescence and enzyme-linked immunosorbent assay. RESULTS Sixty-two patients had pulmonary involvement. Acute respiratory failure developed in nine. Respiratory failure was related to infections in two of them and to ANCA-associated vasculitis in seven. These seven patients uniformly presented with pulmonary hemorrhage and diffuse pulmonary infiltrates. The diagnosis of systemic vasculitis was supported by the presence of a pulmonary-renal syndrome in all patients, and by detection of antibodies to the proteinase 3 or myeloperoxidase antigen in all but one patient. Antiglomerular basement membrane antibodies were absent. The mortality was high due to hypoxic respiratory failure, pulmonary superinfections, and concomitant renal failure. CONCLUSIONS Acute respiratory failure due to vasculitis developed in one of every nine patients with ANCA-associated pulmonary disease. Patients usually present with pulmonary infiltrates and hemoptysis. A diagnosis of vasculitis may be further supported by analysis of the urinary sediment and determination of the ANCA target antigen. It remains to be proved that early detection of ANCA favorably affects the outcome.