Department of Medicine A, Sheba Medical Center, 5262000, Tel Hashomer, Israel.
Danek Gertner Institute of Human Genetics, Tel Hashomer, Israel.
Clin Rheumatol. 2018 May;37(5):1257-1263. doi: 10.1007/s10067-018-4032-3. Epub 2018 Feb 16.
Inflammatory myopathies are a clinically diverse group of diseases, in which the detection of particular autoantibodies may facilitate diagnosis, treatment, and prognosis. The aim of this report is to summarize our experience with specific autoantibody testing in patients with inflammatory myopathies. Data were collected over the last decade in the Autoimmune Center of the Sheba Medical Center, a tertiary referral hospital. Data regarding patients' positive for autoantibodies against Jo-1, PL-7, PL-12, SRP, Mi-2, Ku, and PM-Scl antigens were retrospectively collected. Patient demographics, clinical characteristics, and mortality were recorded. Descriptive statistics (mean, standard deviation, frequency, and percentage) were calculated. A total of 507 patients were surveyed for sclero-poly-synthetase antibodies, as part of the diagnostic workup of myositis/myalgia or interstitial lung disease. Forty-three patients were found positive for one or more of the abovementioned antibodies, and 23 of them (53.49%) had interstitial lung disease (ILD). Four patients were positive for anti-PL-7, three of them had ILD and Raynaud's phenomenon. Five patients were positive for anti-Ku, and four of them had both arthritis and Raynaud's phenomenon. Nine patients were positive for anti-Mi-2, and six of them were given diagnosed with dermatomyositis. Ten patients were positive for anti-SRP, and six of them had cancers of various types. Our results reiterate the previously recognized associations between anti-Mi-2 and dermatomyositis, anti-Ku and Raynaud's phenomenon, and between anti-PL-7 and ILD. In addition, our data support an association between anti-SRP autoantibody positivity and malignancy, which calls for further investigation.
炎性肌病是一组临床表现多样的疾病，其中特定自身抗体的检测有助于诊断、治疗和预后。本报告的目的是总结我们在炎性肌病患者中进行特定自身抗体检测的经验。数据是在过去十年中在谢巴医疗中心的自身免疫中心收集的，该中心是一家三级转诊医院。回顾性收集了抗 Jo-1、PL-7、PL-12、SRP、Mi-2、Ku 和 PM-Scl 抗原自身抗体阳性患者的数据。记录患者的人口统计学、临床特征和死亡率。计算了描述性统计数据（平均值、标准差、频率和百分比）。共有 507 名患者接受了硬皮病多肌炎合成酶抗体检测，作为肌炎/肌痛或间质性肺病诊断的一部分。43 名患者被发现一种或多种上述抗体阳性，其中 23 名（53.49%）患有间质性肺病（ILD）。4 名患者抗 PL-7 阳性，其中 3 名患有 ILD 和雷诺现象。5 名患者抗 Ku 阳性，其中 4 名同时患有关节炎和雷诺现象。9 名患者抗 Mi-2 阳性，其中 6 名被诊断为皮肌炎。10 名患者抗 SRP 阳性，其中 6 名患有各种类型的癌症。我们的结果再次证实了抗 Mi-2 与皮肌炎、抗 Ku 与雷诺现象以及抗 PL-7 与 ILD 之间的先前认识到的关联。此外，我们的数据支持抗 SRP 自身抗体阳性与恶性肿瘤之间的关联，这需要进一步研究。
