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多发性肌炎/皮肌炎中的肺部疾病:65例尸检病例的临床病理分析

Pulmonary disease in polymyositis/dermatomyositis: a clinicopathological analysis of 65 autopsy cases.

作者信息

Lakhanpal S, Lie J T, Conn D L, Martin W J

出版信息

Ann Rheum Dis. 1987 Jan;46(1):23-9. doi: 10.1136/ard.46.1.23.

DOI:10.1136/ard.46.1.23
PMID:3813671
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1002053/
Abstract

The clinical and autopsy records of 65 patients with either polymyositis (24) or dermatomyositis (41) and pulmonary disease were reviewed. Pulmonary symptoms were recorded in 43 of the cases and included dyspnoea in 31, cough in 23, and chest pain in six. Interstitial lung disease was noted at autopsy in 27 patients; almost half of these had arthritis. Bronchopneumonia was found in 35 patients, 31 of these had received prednisone. Dysphagia was present in a similar proportion of patients with and without pneumonia. Pulmonary vasculitis was seen in five patients; pulmonary symptoms, arthritis, and raised erythrocyte sedimentation rate were present in four of these cases and all five had associated interstitial lung disease. Other pulmonary manifestations included pulmonary oedema, primary pulmonary malignancy, diffuse alveolar damage, fibrinous pleuritis, pulmonary emboli, and diaphragmatic atrophy. The mean survival after disease onset was 29 months but was much less for those with interstitial lung disease and pulmonary vasculitis.

摘要

对65例患有多发性肌炎(24例)或皮肌炎(41例)并伴有肺部疾病患者的临床和尸检记录进行了回顾。43例患者记录有肺部症状,包括31例呼吸困难、23例咳嗽和6例胸痛。尸检发现27例患者存在间质性肺疾病;其中近一半患者患有关节炎。35例患者发现支气管肺炎,其中31例曾接受泼尼松治疗。吞咽困难在有肺炎和无肺炎的患者中出现比例相似。5例患者出现肺血管炎;其中4例伴有肺部症状、关节炎和红细胞沉降率升高,且所有5例均伴有间质性肺疾病。其他肺部表现包括肺水肿、原发性肺恶性肿瘤、弥漫性肺泡损伤、纤维素性胸膜炎、肺栓塞和膈肌萎缩。疾病发作后的平均生存期为29个月,但患有间质性肺疾病和肺血管炎的患者生存期要短得多。

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