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并发于结节病的慢性肺曲霉病。

Chronic pulmonary aspergillosis complicating sarcoidosis.

机构信息

Paris 13 University, EA2363, Sorbonne Paris Cité, Bobigny, France

Assistance Publique - Hôpitaux de Paris, Avicenne Hospital, Pulmonary Dept, Bobigny, France.

出版信息

Eur Respir J. 2017 Jun 15;49(6). doi: 10.1183/13993003.02396-2016. Print 2017 Jun.

DOI:10.1183/13993003.02396-2016
PMID:28619957
Abstract

Chronic pulmonary aspergillosis (CPA) complicating sarcoidosis (SA) is associated with high mortality, and there is a lack of clarity regarding the relative contributions of SA or CPA.This was a retrospective single-centre study on CPA-SA.In total, 65 patients (44 men), aged 41.4±13.5 and 48.3±11.9 years at the time of SA and CPA diagnoses, respectively, were included between 1980 and 2015. Of these, 64 had fibrocystic SA, most often advanced, with composite physiological index (CPI) >40 (65% of patients) and pulmonary hypertension (PH) (31%), and 41 patients (63%) were treated for SA (corticosteroids or immunosuppressive drugs). Chronic cavitary pulmonary aspergillosis (CCPA) was the most frequent CPA pattern. Regarding treatment, 55 patients required long-term antifungals, 14 interventional radiology, 11 resection surgery and two transplantation. Nearly half of the patients (27; 41.5%) died (mean age 55.8 years); 73% of the patients achieved 5-year survival and 61% 10-year survival. Death most often resulted from advanced SA and rarely from haemoptysis. CPI, fibrosis extent and PH predicted survival. Comparison with paired healthy controls without CPA did not show any difference in survival, but a higher percentage of patients had high-risk mould exposure.CPA occurs in advanced pulmonary SA. CPA-SA is associated with high mortality due to the underlying advanced SA rather than to the CPA. CPI, fibrosis extent and PH best predict outcome.

摘要

慢性肺曲霉病(CPA)并发结节病(SA)与高死亡率相关,对于 SA 或 CPA 的相对贡献尚不清楚。这是一项回顾性单中心研究,研究对象为 CPA-SA。1980 年至 2015 年间共纳入 65 例患者(44 名男性),SA 和 CPA 诊断时的年龄分别为 41.4±13.5 岁和 48.3±11.9 岁。其中,64 例为纤维囊性 SA,多为晚期,复合生理指数(CPI)>40(65%的患者)和肺动脉高压(PH)(31%),41 例(63%)因 SA 接受治疗(皮质类固醇或免疫抑制剂)。慢性空洞性肺曲霉病(CCPA)是最常见的 CPA 类型。关于治疗,55 例患者需要长期使用抗真菌药物,14 例需要介入放射学治疗,11 例需要手术切除,2 例需要移植。近一半的患者(27 例;41.5%)死亡(平均年龄 55.8 岁);73%的患者 5 年生存率和 61%的患者 10 年生存率。死亡最常见于晚期 SA,很少由咯血引起。CPI、纤维化程度和 PH 预测生存率。与没有 CPA 的配对健康对照组相比,死亡率没有差异,但高危霉菌暴露的患者比例更高。CPA 发生于晚期肺部 SA。由于潜在的晚期 SA,而不是 CPA,CPA-SA 与高死亡率相关。CPI、纤维化程度和 PH 是最佳的预后预测因素。

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