副肿瘤相关性风湿综合征

Paraneoplastic Syndrome in Rheumatic Disorders

副肿瘤相关性风湿综合征指恶性肿瘤模拟风湿性疾病,以风湿样综合征为其突出表现,且这些风湿样表现与肿瘤的直接浸润或转移无关。风湿样表现可以早于、同步于或晚于恶性肿瘤的其他症状。当风湿样表现早于或同步于恶性肿瘤的其他症状时,往往早期识别困难,可能错失最佳诊治时机,因此提高认识,加强风湿病与肿瘤的鉴别诊断非常重要。本文通过回顾文献,总结副肿瘤相关性风湿综合征的临床特点及治疗,如老年人出现不典型的风湿病样表现,包括肥厚性骨关节病、副肿瘤性多关节炎、血清阴性滑膜炎伴凹陷性水肿综合征、掌筋膜炎及关节炎、红皮病、副肿瘤天疱疮、远端肌无力或肌痛、急性坏死性肌病等,或对于典型风湿病症状者出现以下难以解释的表现,包括体重减轻、纳差、不明原因发热等恶病质表现,网状内皮系统增殖表现,不明原因包块,肿瘤标志物升高,缺乏特异性免疫学指标,常规治疗效果不佳等情况时需高度警惕潜在恶性肿瘤,加强肿瘤相关筛查及随访。副肿瘤相关性风湿综合征的出现或复发预示着原发肿瘤的复发或转移,应以治疗原发肿瘤为主。

Paraneoplastic syndrome in rheumatic disorders is defined by clinical, radiological or biological manifestations associated with malignant diseases, but without direct invasion by the tumor. Rheumatoid manifestations may coincide,precede or follow the diagnosis of tumor,or herald its recurrence. The clinical course generally parallels that of the primary tumor, and treatment of the underlying malignancy often accompanies regression of the rheumatic disorders. It is difficult for rheumatologist to recognize it when rheumatoid manifestations coincide or precede the occurrence of malignancy. Thus caution is required. We reviewed the literatures about paraneoplastic syndrome in rheumatic disorders and concluded its manifestations. Rheumatic manifestations related with malignancies are usually atypical. For instance,Hypertrophic osteoarthropathy, Paraneoplastic polyarthritis, Remitting seronegative symmetrical synovitis with pitting oedema,Palmar fasciitis and polyarthritis syndrome,erythroderma,paraneoplastic pemphigus,distal myalgia or muscle weakness, acute necrotizing myopathy, and so on. For patients with typical rheumatoid symptoms, it should be considered for additional investigations to rule out a diagnosis ofmalignancy for those unexplained symptoms： cachexia,proliferation of reticuloendothelial system,lumps due to unknown reason,high tumor markers,absent autoimmune antibodies,poor therapeutic effect,and so on. All those should alert rheumatologists the possibility of occult malignancies. If paraneoplastic syndrome in rheumatic disorders are diagnosed,the treatment of tumor is more urgent,instead of corticosteroid and immunosuppressant.