Chatté G, Boyer J
Service de Pneumologie, Centre hospitalier Lucien Hussel, Vienne.
Rev Mal Respir. 1992;9(6):634-5.
The authors describe a case of interstitial lung disease associated with dermatomyositis. Alveolar-interstitial shadowing on the initial chest X-ray, lymphocytosis on the broncho-alveolar lavage fluid, absence of anti-JO1 antibodies and presence of intra-alveolar buds on the open lung biopsy were observed. Our patient died of acute respiratory failure, 34 months after symptoms onset. Intra-alveolar buds found at an early stage of dermatomyositis interstitial lung disease, were associated with minimal interstitial cellularity, markedly interstitial fibrosis, poor prognosis and poor response to steroid therapy. In contrary, bronchiolitis obliterans organizing pneumonia noted at an early stage of interstitial lung disease associated with both dermatomyositis and polymyositis, seems to have better prognosis and response to corticosteroid, especially if associated with polymyositis.
作者描述了一例与皮肌炎相关的间质性肺疾病。在初始胸部X光片上观察到肺泡-间质阴影，支气管肺泡灌洗液中淋巴细胞增多，抗JO1抗体阴性，开胸肺活检显示肺泡内芽肿。我们的患者在症状出现34个月后死于急性呼吸衰竭。在皮肌炎间质性肺疾病早期发现的肺泡内芽肿，与间质细胞极少、明显的间质纤维化、预后不良以及对类固醇治疗反应不佳有关。相反，在与皮肌炎和多肌炎相关的间质性肺疾病早期发现的闭塞性细支气管炎伴机化性肺炎，似乎预后较好，对皮质类固醇治疗反应良好，尤其是与多肌炎相关时。
