Clinical and serological characteristics of 125 Dutch myositis patients - Myositis specific autoantibodies aid in the differential diagnosis of the idiopathic inflammatory myopathies
Clinical and serological characteristics of 125 Dutch myositis patients - Myositis specific autoantibodies aid in the differential diagnosis of the idiopathic inflammatory myopathies
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DOI：
10.1007/pl00007850
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发表时间：
2002-01-01
影响因子：
6
通讯作者：
van Engelen, BGM
中科院分区：
文献类型：
--
作者：
Hengstman, GJD;Brouwer, R;van Engelen, BGM
特发性炎症性肌病 (IIM) 是一组异质性全身性疾病，包括常见的皮肌炎 (DM)、多发性肌炎 (PM) 和包涵体肌炎 (IBM)。一部分患者具有 IIM 特异性的独特自身抗体（肌炎特异性自身抗体；MSA）。我们研究了 125 名荷兰患者的 IIM 临床和血清学特征。通过免疫印迹、酶联免疫吸附测定和免疫沉淀分析血清。最常见的 MSA 是抗 Jo-1 自身抗体 (20%)，其次是抗 tRNA(His) (6%)、抗 Mi-2 (6%) 和抗 SRP (4%)。某些 MSA 的存在显然与特定的临床特征相关。抗 Jo-1 和抗 tRNA (His) 与抗合成酶综合征、抗 SRP 和 PM 相关，伴有严重的肌痛和关节痛以及对免疫抑制治疗的中度反应。一个新的发现是抗 Mi-2 的存在，不仅存在于 DM 中，而且存在于 PM 中。 MSA 经常存在于 DM/PM 血清中，但在 IBM 患者的血清中几乎未检测到。少数患有 MSA 的 IBM 患者表现出对免疫抑制治疗的显着反应。可以得出结论，MSA 定义了 IIM 范围内的特定临床综合征，并且它们可以通过实际上排除 IBM 的存在，并通过潜在地识别类固醇反应性 IBM 患者的亚组，来协助这些神秘疾病的鉴别诊断和治疗计划。
The idiopathic inflammatory myopathies (IIM) are a heterogeneous group of systemic diseases that include the familiar disease entities of dermatomyositis (DM), polymyositis (PM), and inclusion body myositis (IBM). A subset of patients has unique autoantibodies which are specific for IIM (myositis specific autoantibodies; MSAs). We studied the clinical and serological characteristics of IIM in 125 Dutch patients. Sera were analysed by immunoblotting, enzyme-linked immunosorbent assay, and immunoprecipitation. The most frequently encountered MSA was the anti-Jo-1 autoantibody (20%), followed by anti-tRNA(His) (6%), anti-Mi-2 (6%), and anti-SRP (4%). The presence of certain MSAs was clearly associated with specific clinical characteristics. Anti-Jo-1 and anti-tRNA(His) were associated with the anti-synthetase syndrome, anti-SRP with PM with severe myalgia and arthralgia and a moderate response to immunosuppressive treatment. A novel finding was the presence of anti-Mi-2, not only in DM, but also in PM. MSAs were frequently present in DM/PM sera, but were hardly ever detected in the sera of IBM patients. The few IBM patients with MSAs demonstrated a significant response to immuno suppressive treatment. It can be concluded that MSAs define specific clinical syndromes within the spectrum of IIM and that they can assist in the differential diagnosis and treatment plan of these enigmatic disorders by virtually excluding IBM by their presence, and by potentially identifying a subgroup of steroid-responsive IBM patients.