华西医学

华西医学

特发性炎性肌病相关的间质性肺疾病

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特发性炎性肌病是一类以横纹肌非化脓性炎症为特征的结缔组织病。目前临床上主要沿用 Bohan 和 Peter 于 1976 年提出的多发性肌炎/皮肌炎分类诊断标准。炎性肌病不仅累及皮肤和肌肉,也累及全身多个系统,其中累及肺部导致的间质性肺疾病(interstitial lung diseases,ILD)占据了重要部分,同时 ILD 也是炎性肌病患者死亡的重要原因。对特发性炎性肌病患者需尽早行胸部高分辨率 CT 检查,提高 ILD 的检出率,尽早进行干预治疗。因为肌炎患者血清中肌炎特异性抗体的不同,临床表现不一,部分肌炎特异性抗体可提示患者合并 ILD,并且预测患者疾病发展过程。如抗合成酶抗体阳性的患者,其 ILD 的发病率较高,抗黑素瘤分化相关基因 5 抗体则与快速进展、预后差的 ILD 明显相关。及时筛查出肌炎特异性抗体阳性的 ILD 患者,可以对患者整个疾病的诊治过程有所帮助。

Idiopathic inflammatory myopathies are a group of connective tissue diseases characterized by nonsuppurative inflammation of the striated muscle. At present, the diagnostic criteria for polymyositis/dermatomyositis classification proposed by Bohan and Peter in 1976 is mainly used clinically. In clinical observations, it is found that myopathy involves not only skin and muscle but also affects many systems of the body. Interstitial lung disease occupies an important part, and it is an important cause of death of patients with inflammatory myopathy. Patients with idiopathic myositis should be examined as early as possible by high-resolution CT to improve the detection rate of myositis-associated interstitial lung disease and start treatment as soon as possible. At the same time, the patients with myositis have different clinical manifestations due to specific antibodies in the serum; some specific antibodies may indicate poor prognosis and poor treatment response. Timely screening of patients with positive myositis-specific antibodies in patients with the pulmonary interstitial disease can help the patient’s diagnosis and treatment process.

关键词: 特发性炎性肌病; 肌炎特异性抗体; 间质性肺疾病; 肌炎相关间质性肺疾病

Key words: Idiopathic inflammatory myopathies; Myositis-specific autoantibodies; Interstitial lung diseases; Inflammatory myopathies associated interstitial lung disease

引用本文: 胡译丹, 刘钢, 谢其冰. 特发性炎性肌病相关的间质性肺疾病. 华西医学, 2018, 33(12): 1544-1548. doi: 10.7507/1002-0179.201805070 复制

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