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首页> 外文期刊>Journal of clinical rheumatology >Microscopic polyangiitis: A large single-center series
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Microscopic polyangiitis: A large single-center series

机译：显微镜下多血管炎：大型单中心系列

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Objective: Microscopic polyangiitis (MPA) is a rare systemic vasculitis, antineutrophil cytoplasmic antibody associated, characterized by necrotizing small vessel involvement with few or no immune complex deposits. Necrotizing glomerulonephritis is common. Pulmonary capillaritis causing alveolar hemorrhage and hemoptysis is well recognized, but most case series are reported in the nephrology literature and emphasize renal considerations. We present a single-center 10-year retrospective review of 40 patients meeting the 2012 Revised Chapel Hill Nomenclature of MPA, with specific p-antineutrophil cytoplasmic antibody and myeloperoxidase positivity, emphasizing initial and subsequent pulmonary manifestations. Methods: We searched the electronic database of our large integrated clinic-hospital system and reviewed charts of all patients with related International Classification of Diseases, Ninth Revision codes for vasculitis in the last 10 years. Several variables were reviewed. Results: Onset of illness was usually abrupt and included respiratory symptoms, and the most common presenting complaint was cough. Hemoptysis occurred during the course of illness in 40%. Pulmonary complaints were found in 80% of patients upon presentation, whereas pulmonary infiltrates were noted in 92%. Managing subspecialty and treatment modalities were quite variable. Conclusions: Pulmonary involvement is much more frequent than the currently reported 25% to 50% when features in addition to hemorrhage are recorded. No clear guidelines direct the evaluation and management of MPA patients. Consistent communication between pulmonary, nephrology, and rheumatology services could improve our understanding of the disease process.
机译：目的：镜下性多发性血管炎（MPA）是一种罕见的全身性血管炎，是抗中性粒细胞胞浆相关抗体，其特征是坏死小血管，几乎没有免疫复合物沉积。坏死性肾小球肾炎很常见。引起肺泡出血和咯血的肺毛细血管炎已广为人知，但大多数病例系列在肾脏病学文献中都有报道，并强调了肾脏方面的考虑。我们对40名符合2012年修订的MPA Chapel Hill命名法的患者进行了单中心10年回顾性审查，这些患者具有特异性的p-抗中性粒细胞胞浆抗体和髓过氧化物酶阳性，强调了最初和随后的肺部表现。方法：我们搜索了大型综合医院-医院系统的电子数据库，并回顾了过去十年中所有具有相关国际疾病分类，第九版血管炎修订代码的患者的图表。审查了几个变量。结果：疾病发作通常是突然的，包括呼吸道症状，最常见的主诉是咳嗽。咯血发生在病程中的占40％。在就诊时发现有80％的患者出现肺部不适，而在92％的患者中发现了肺浸润。亚专科治疗和治疗方式变化很大。结论：当记录除了出血以外的其他特征时，肺部受累比目前报道的25％至50％更为频繁。没有明确的指导方针指导MPA患者的评估和治疗。肺，肾病和风湿病服务之间的一致沟通可以增进我们对疾病过程的了解。

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