Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis
Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis
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DOI：
10.1183/09031936.03.00026703
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发表时间：
2003-08-01
影响因子：
24.3
通讯作者：
Cordier, JF
中科院分区：
文献类型：
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作者：
Cottin, V;Thivolet-Béjui, F;Cordier, JF
本研究调查了与淀粉样皮肌炎、皮肌炎和多发性肌炎相关的间质性肺炎，特别关注肌肉和/或皮肤表现及其与肺部累及的时间顺序。患者男性4例，女性13例，年龄51.7±10.8岁，均行手术肺活检。诊断包括皮肌炎（10例）、多发性肌炎（4例）和淀粉性皮肌炎（3例）。4例（24%）患者在出现任何皮肤或肌肉疾病之前有单独的呼吸症状。网状和磨玻璃混浊是最常见的CT表现。病理检查显示非特异性间质性肺炎（11例，65%；细胞性，2例；细胞性和纤维化性，5例；纤维化性，4例），常规间质性肺炎（2例），组织性肺炎（2例），淋巴细胞性间质性肺炎（1例），无法分型的间质性肺炎（1例）。非特异性间质性肺炎是淀粉样皮肌炎患者（3 / 3）和以呼吸道症状为结缔组织病初始临床表现的患者（3 / 4）中间质性肺炎最常见的组织学类型。5年生存率为50%。本研究显示，临床医生在做出非特异性间质性肺炎的病理诊断时，应警惕潜在的肌肉或皮肤表现。
This study investigated interstitial pneumonia associated with amyopathic dermatomyositis, dermatomyositis and polymyositis, paying particular attention to muscular and/or cutaneous manifestations and their chronology relative to lung involvement. Patients included four males and 13 females, aged 51.7+/-10.8 yrs, who had surgical lung biopsy.Diagnoses included dermatomyositis (10 patients), polymyositis (four patients) and amyopathic dermatomyositis (three patients). Solitary respiratory manifestations preceded the onset of any skin or muscle disease in four cases (24%). Reticular and ground glass opacities were the most frequent computed tomography (CT) findings. Pathological review showed nonspecific interstitial pneumonia (eleven, 65%; cellular, two; cellular and fibrotic, five; fibrotic, four), usual interstitial pneumonia (two), organising pneumonia (two), lymphocytic interstitial pneumonia (one), and unclassifiable interstitial pneumonia (one). Nonspecific interstitial pneumonia was the most common histological pattern of interstitial pneumonia in patients with amyopathic dermatomyositis (three of three) and in patients with respiratory symptoms as the initial clinical manifestation of the connective tissue disease (three of four). Survival at 5 yrs was 50%.This study shows the clinician should remain alert to potential muscular or cutaneous manifestations whenever a pathological diagnosis of nonspecific interstitial pneumonia is made.