Tillie-Leblond I, Colin G, Lelong J, Cadranel J
Service de Pneumologie et d'Immuno-Allergologie, Hôpital Calmette, Lille, France.
Rev Mal Respir. 2006 Dec;23(6):671-80. doi: 10.1016/s0761-8425(06)72081-2.
Polymyositis is characterised by an inflammatory reaction in skeletal muscle with a variable degree of muscular weakness and associated with skin lesions in the case of dermatomyositis. Involvement of the muscles of deglutition and the diaphragm may lead to inhalation pneumonia and acute or chronic respiratory failure, often hypercapnic. The other respiratory manifestations are diffuse interstitial pneumonitis (DIP), usually non-specific, and very occasionally pulmonary arterial hypertension. The development of DIP during polymyositis is a grave prognostic factor, respiratory involvement being one of the main causes of morbidity and mortality. The onset of DIP is acute in between 30 and 47% of cases. Anti-synthetase antibodies (particularly anti-JO-1) are positive in about 75% of cases. Treatment is usually with a combination of immunosuppressants and corticosteroids without any immunosuppressants therapy having shown a superiority.
多发性肌炎的特征是骨骼肌出现炎症反应,伴有不同程度的肌肉无力,在皮肌炎的情况下还伴有皮肤病变。吞咽肌和膈肌受累可能导致吸入性肺炎以及急性或慢性呼吸衰竭,通常为高碳酸血症性呼吸衰竭。其他呼吸系统表现为弥漫性间质性肺炎(DIP),通常不具有特异性,极个别情况下会出现肺动脉高压。多发性肌炎期间DIP的发生是一个严重的预后因素,呼吸系统受累是发病和死亡的主要原因之一。30%至47%的病例中DIP起病急性。约75%的病例抗合成酶抗体(尤其是抗JO-1)呈阳性。治疗通常采用免疫抑制剂和皮质类固醇联合使用,尚无任何一种免疫抑制剂疗法显示出优越性。