Benbassat J, Gefel D, Larholt K, Sukenik S, Morgenstern V, Zlotnick A
Arthritis Rheum. 1985 Mar;28(3):249-55. doi: 10.1002/art.1780280303.
An effort was made to identify all patients with polymyositis/dermatomyositis (PM/DM) admitted to hospitals in Israel from 1956-1976. The diagnosis of PM/DM was retrospectively reviewed in 92 (46 definite, 26 probable, and 20 possible) cases. The most common complaints and physical findings in the course of the disease were muscle weakness (86 patients), rash (53 patients), arthritis or arthralgia (39 patients), and dysphagia (35 patients). Elevated serum aldolase levels were found in 64% of the patients for whom data were available; 92% had abnormal electromyogram results, and 60.9% had muscle histopathology consistent with PM/DM. Malignancy was diagnosed in 13 patients. Malignancy, ischemic heart disease, and pulmonary complications were the most common causes of death. The actuarial survival curve was heterogeneous, with an accelerated mortality during the first year after diagnosis and a slower mortality during the following 7 years. Independent unfavorable prognostic signs were: failure to induce remission, leukocytosis, fever, older age, a shorter disease history, and dysphagia.
研究人员致力于识别1956年至1976年期间入住以色列医院的所有多发性肌炎/皮肌炎(PM/DM)患者。对92例(46例确诊、26例疑似和20例可能)患者的PM/DM诊断进行了回顾性分析。该疾病过程中最常见的症状和体征为肌无力(86例患者)、皮疹(53例患者)、关节炎或关节痛(39例患者)以及吞咽困难(35例患者)。在有数据可查的患者中,64%的患者血清醛缩酶水平升高;92%的患者肌电图结果异常,60.9%的患者肌肉组织病理学结果符合PM/DM。13例患者被诊断为恶性肿瘤。恶性肿瘤、缺血性心脏病和肺部并发症是最常见的死亡原因。精算生存曲线具有异质性,诊断后的第一年死亡率加速上升,随后7年死亡率上升较慢。独立的不良预后指标包括:无法诱导缓解、白细胞增多、发热、年龄较大、病程较短以及吞咽困难。