Idiopathic inflammatory myopathies and the lung.
Idiopathic inflammatory myopathies and the lung.
复制标题
DOI：
10.1183/16000617.00002015
复制
发表时间：
2015-06
影响因子：
7.5
通讯作者：
Cottin, Vincent
中科院分区：
文献类型：
--
作者：
Lega, Jean-Christophe;Reynaud, Quitterie;Belot, Alexandre;Fabien, Nicole;Durieu, Isabelle;Cottin, Vincent
特发性炎症性肌炎（IIM）是一组罕见的结缔组织疾病（CTDs），其特征是肌肉和肌外体征，其中肺部受累是一个具有挑战性的问题。间质性肺疾病（ILD）是IIM肺部受累的标志，并导致发病率和死亡率，在一些系列中估计死亡率超过50%。除包涵体肌炎外，这些肺外疾病与其他CTD中常见的全身和内脏受累相关，包括发热、雷诺现象、关节痛、非特异性皮肤改变和ILD，其患病率估计高达65%。在IIM的谱内存在实质性异质性，并且每种状况与肺部受累的不同频率和亚型相关。这种异质性部分与包括抗合成酶、抗MDA 5和抗PM/Scl在内的各种自身抗体的存在有关。ILD存在于IIM的所有子集中，包括幼年型肌炎，但在皮肌炎和重叠型肌炎中更常见。IIM也可能与其他呼吸系统受累相关，即肺动脉高压、胸膜疾病、感染、药物诱导的毒性、恶性肿瘤和呼吸肌无力。在这里，我们批判性地回顾了目前关于成人和青少年肌炎相关肺部疾病的知识，并详细描述了慢性和快速进展性ILD的治疗方法。间质性肺病是皮肌炎/多发性肌炎发病的主要原因http://ow.ly/M1wB8
Idiopathic inflammatory myositis (IIM) is a group of rare connective tissue diseases (CTDs) characterised by muscular and extramuscular signs, in which lung involvement is a challenging issue. Interstitial lung disease (ILD) is the hallmark of pulmonary involvement in IIM, and causes morbidity and mortality, resulting in an estimated excess mortality of 50% in some series. Except for inclusion body myositis, these extrapulmonary disorders are associated with the general and visceral involvement frequently found in other CTDs including fever, Raynaud's phenomenon, arthralgia, nonspecific cutaneous modifications and ILD, for which the prevalence is estimated to be up to 65%. Substantial heterogeneity exists within the spectrum of IIMs, and each condition is associated with various frequencies and subtypes of pulmonary involvement. This heterogeneity is partly related to the presence of various autoantibodies encompassing anti-synthetase, anti-MDA5 and anti-PM/Scl. ILD is present in all subsets of IIM including juvenile myositis, but is more frequent in dermatomyositis and overlap myositis. IIM can also be associated with other presentations of respiratory involvement, namely pulmonary arterial hypertension, pleural disease, infections, drug-induced toxicity, malignancy and respiratory muscle weakness. Here, we critically review the current knowledge about adult and juvenile myositis-associated lung disease with a detailed description of therapeutics for chronic and rapidly progressive ILD. Interstitial lung disease is a leading cause of morbidity in dermatomyositis/polymyositis http://ow.ly/M1wB8