Fer F, Allenbach Y, Benveniste O
Département de Médecine interne et immunologie clinique, Centre national de référence des maladies neuromusculaires, hôpital Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France.
Département de Médecine interne et immunologie clinique, Centre national de référence des maladies neuromusculaires, hôpital Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France.
Rev Med Interne. 2021 Jun;42(6):392-400. doi: 10.1016/j.revmed.2020.10.379. Epub 2020 Nov 25.
Idiopathic inflammatory myopathies, or IIM, are a group of acquired diseases that affect the muscle to a certain extent, and may also affect other organs. They include dermatomyositis, which can affect the muscle eventualy, with a typical skin rash; inclusion body myositis, with a purely muscular expression resulting in a slow progressive deficit; and the former group of "polymyositis", a misnomer that actually includes other categories of IIM, such as immune-mediated necrotizing myopathies, with a severe muscle involvement often presents from the onset of the disease; antisynthetase syndrome, which combines muscle damage, joint involvement and a potentially life-threatening lung disease; and overlapping myositis, which combines muscle damage with other organs involvement connected to another autoimmune disease. The diagnosis of IIM is based on rigorous clinical examination and interrogation, electromyographic data and immunological testing for myositis specific antibodies. This antibody dosage must be extended or repeated if necessary to classify correctly the muscle disease under investigation, as the available tests may not perform well enough. Muscle biopsy, although very informative, is not anymore systematically recommended when the clinic and the antibodies are typical. However, some forms of IIM are sometimes difficult to classify; in these cases, muscle biopsy plays a crucial role in the precise etiological diagnosis.
特发性炎性肌病(IIM)是一组后天性疾病,会在一定程度上影响肌肉,也可能影响其他器官。它们包括皮肌炎,最终可影响肌肉,并伴有典型皮疹;包涵体肌炎,仅表现为肌肉症状,导致缓慢进展性功能障碍;以及前一组“多发性肌炎”,这是一个误称,实际上包括其他类型的IIM,如免疫介导的坏死性肌病,疾病发作时通常会出现严重的肌肉受累;抗合成酶综合征,伴有肌肉损伤、关节受累以及可能危及生命的肺部疾病;重叠性肌炎,将肌肉损伤与与另一种自身免疫性疾病相关的其他器官受累相结合。IIM的诊断基于严格的临床检查和问诊、肌电图数据以及针对肌炎特异性抗体的免疫检测。如有必要,必须扩大或重复这种抗体检测,以便正确分类所研究的肌肉疾病,因为现有的检测可能不够准确。肌肉活检虽然信息丰富,但当临床表现和抗体检测结果典型时,不再系统推荐进行此项检查。然而,某些形式的IIM有时难以分类;在这些情况下,肌肉活检在精确的病因诊断中起着关键作用。