目的：探讨系统性硬化症和恶性肿瘤之间的关系，提高临床医师对此病的认识。方法收集1992年2月至2015年2月在北京协和医院住院的系统性硬化症并发恶性肿瘤的患者共39例，分析其临床表现、实验室检查、影像学检查、病理学检查及治疗。结果39例系统性硬化症患者中局限皮肤型7例，弥漫皮肤型32例，肿瘤先行发生3例，同时发生6例，肿瘤后续发生30例。并发恶性肿瘤17种，其中以呼吸系统和生殖系统肿瘤最为常见。系统性硬化症出现肺间质病变22例，肺动脉高压8例，心包积液8例，胃肠道反流14例，蛋白尿5例，血液系统改变12例，并发肌炎5例。结论系统性硬化症可并发多种恶性肿瘤，重视早期筛查有助于疾病的检出和治疗。%Objective To investigate the relationship between systemic sclerosis and tumor.Methods The data of 39 Systemic sclerosis patients with malignant tumor from Peking Union Medical College Hospital from February 1992 to February 2015 were collected.The clinical manifestation, laboratory examinations, imaging presentations, pathological examination and treatment were summarized.Results Thirty-two diffuse cutaneous SSc ( dcSSc ) patients and 7 limited cuta neous SSc ( lcSSc) patients were identified complicated with malignancy.Three cancers were diagnosed before the onset of SSc, 6 cancers were at the same time, 30 were after the onset of SSc.Eighteen kinds of tumors were diagnosed in which respiratory and reproductive system tumors were the most common.Twenty-two patients had interstitial lung disease, 5 had proteinuria, 12 had hematological system involvements, 5 had myositis. Conclusion Systemic sclerosis could complicate with a variety of neoplastic diseases, early screening is helpful to the diagnosis and treatment of tumor.
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