Bartosiewicz Małgorzata, Siemion-Szcześniak Izabela, Sobiecka Małgorzata, Wyrostkiewicz Dorota, Radwan-Röhrenschef Piotr, Lewandowska Katarzyna, Langfort Renata, Oniszh Karina, Franczuk Monika, Kuś Jan
I Klinika Chorób Płuc, Instytut Gruźlicy i Chorób Płuc w W arszawie.
Pneumonol Alergol Pol. 2013;81(6):556-66.
Polymyositis (PM) and dermatomyositis (DM) are connective tissue diseases (CTD) characterized by proximal muscle weakness along with changes in various internal organs, with the lungs most frequently involved. Presentation of the disease in the lungs comprises diffuse alveolar haemorrhage due to vasculitis and interstitial lung disease (ILD), which is the most frequent manifestation of CTD in the lungs and worsens the outcome and prognosis. The mechanisms involved in the ILD are not fully known, but the role of autoimmune response is unquestioned. No relationship between the severity of CTD and the changes in the lungs was observed. ILD may present at any time in the course of CTD, sometimes before the signs and symptoms of myositis occur. The more accurate imaging methods are, the more frequently changes in the lungs are detected. High resolution computed tomography (HRCT) is a gold standard in ILD imaging. Treatment of PM/DM-related ILD relays on systemic glucocorticosteroids as the first choice drugs. We present three cases of PM/DM-related ILD in middle-aged men, with a different clinical and radiological presentation. In all cases, apart from imaging (plain X-ray and HRCT of the chest) and pulmonary function tests, histological evaluation of lung changes was performed. In two cases non-specific interstitial pneumonitis (NSIP) was diagnosed, and in the third--organizing pneumonia along with sarcoid changes in the lymph nodes. Because of decreased pulmonary function all patients were treated with systemic corticosteroids and two of them additionally with azathioprine or cyclophosphamide, and the outcome was good in all of them.
多发性肌炎(PM)和皮肌炎(DM)是结缔组织病(CTD),其特征为近端肌无力以及各种内脏器官的改变,其中肺部受累最为常见。该疾病在肺部的表现包括血管炎导致的弥漫性肺泡出血和间质性肺疾病(ILD),ILD是CTD在肺部最常见的表现,会使病情转归和预后恶化。ILD的发病机制尚未完全明确,但自身免疫反应的作用是毋庸置疑的。未观察到CTD的严重程度与肺部改变之间存在关联。ILD可能在CTD病程中的任何时候出现,有时在肌炎的体征和症状出现之前。成像方法越精确,肺部改变的检出率就越高。高分辨率计算机断层扫描(HRCT)是ILD成像的金标准。PM/DM相关ILD的治疗首选全身糖皮质激素。我们报告了3例中年男性PM/DM相关ILD病例,其临床和影像学表现各异。在所有病例中,除了进行影像学检查(胸部平片和HRCT)及肺功能测试外,还对肺部改变进行了组织学评估。2例诊断为非特异性间质性肺炎(NSIP),第3例诊断为机化性肺炎伴淋巴结结节病样改变。由于肺功能下降,所有患者均接受了全身糖皮质激素治疗,其中2例还加用了硫唑嘌呤或环磷酰胺,所有患者预后良好。