Ponyi Andrea, Constantin Tamás, Garami Miklós, András Csilla, Tállai Béla, Váncsa Andrea, Gergely Lajos, Dankó Katalin
Division of Clinical Immunology, Third Department of Internal Medicine, Medical and Health Science Center, University of Debrecen, Moricz Zs Krt 22, 4004 Debrecen, Hungary.
Ann N Y Acad Sci. 2005 Jun;1051:64-71. doi: 10.1196/annals.1361.047.
Idiopathic inflammatory myositis is characterized by progressive weakness of the proximal muscles. There is a higher risk of malignancy than in the normal population. The aim of this study was to evaluate the frequency of malignancy among 251 myositis patients. We also compared clinical and immunological characteristics of cancer-associated myositis with primary myositis. There were no malignancies among polymyositis, overlap, or juvenile myositis patients. Twenty-two of ninety dermatomyositis patients also had a malignant disease. Patients with cancer-associated dermatomyositis were significantly older than primary myositis patients and had more severe cutaneous and muscle symptoms. Dysphagia and diaphragmatic involvement were more frequent among cancer-associated patients, while extramuscular features were less frequent. After successful treatment of the malignancy, we were able to manage myositis symptoms. One-year survival rate was significantly better in primary dermatomyositis patients. The subset of cancer-associated myositis differs from primary myositis in many aspects of its clinical and immunological features. Prognosis and life expectancy in cancer-associated myositis patients is determined by the underlying malignant disease. Therefore, age- and sex-specific examinations for detection of an underlying malignancy are important in the management of patients with dermatomyositis.
特发性炎性肌病的特征是近端肌肉进行性无力。其患恶性肿瘤的风险高于正常人群。本研究的目的是评估251例肌炎患者中恶性肿瘤的发生率。我们还比较了癌症相关性肌炎与原发性肌炎的临床和免疫学特征。多发性肌炎、重叠综合征或青少年肌炎患者中未发现恶性肿瘤。90例皮肌炎患者中有22例也患有恶性疾病。癌症相关性皮肌炎患者的年龄显著大于原发性肌炎患者，且皮肤和肌肉症状更严重。吞咽困难和膈肌受累在癌症相关性患者中更常见，而肌肉外表现则较少见。恶性肿瘤成功治疗后，我们能够控制肌炎症状。原发性皮肌炎患者的1年生存率明显更好。癌症相关性肌炎亚组在临床和免疫学特征的许多方面与原发性肌炎不同。癌症相关性肌炎患者的预后和预期寿命取决于潜在的恶性疾病。因此，针对潜在恶性肿瘤的年龄和性别特异性检查在皮肌炎患者的管理中很重要。
