Cottin V, Thivolet-Béjui F, Reynaud-Gaubert M, Cadranel J, Delaval P, Ternamian P J, Cordier J F
Dept of Respiratory Medicine, Louis Pradel Hospital, Claude Bernard University, Lyon, France.
Eur Respir J. 2003 Aug;22(2):245-50. doi: 10.1183/09031936.03.00026703.
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.
本研究调查了与无肌病性皮肌炎、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受累的时间顺序。患者包括4名男性和13名女性，年龄51.7±10.8岁，均接受了外科肺活检。诊断包括皮肌炎（10例）、多发性肌炎（4例）和无肌病性皮肌炎（3例）。4例（24%）患者在出现任何皮肤或肌肉疾病之前先出现孤立的呼吸系统表现。网状和磨玻璃影是计算机断层扫描（CT）最常见的表现。病理检查显示非特异性间质性肺炎（11例，65%；细胞型2例；细胞型和纤维化型5例；纤维化型4例）、寻常型间质性肺炎（2例）、机化性肺炎（2例）、淋巴细胞性间质性肺炎（1例）和无法分类的间质性肺炎（1例）。非特异性间质性肺炎是无肌病性皮肌炎患者（3例中的3例）以及以呼吸道症状为结缔组织病初始临床表现的患者（4例中的3例）中间质性肺炎最常见的组织学类型。5年生存率为50%。本研究表明，每当做出非特异性间质性肺炎的病理诊断时，临床医生应警惕潜在的肌肉或皮肤表现。
