成人斯蒂尔病112例临床分析

Clinical analysis on 112 patients of adult onset Still's disease

目的:探讨成人斯蒂尔病(adult-onsetStill's disease,AOSD)的临床特征、诊断、治疗与转归,以提高对该病的认识。方法:对112例AOSD的临床表现、实验室检查、治疗和预后等临床资料进行数理分析。结果:发热、关节痛、咽痛、皮疹和肌肉疼痛是AOSD患者最常见的5个症状。呼吸系统受累多见(33%),其中间质性肺疾病5例,其次为循环系统(17%),消化系统(7%),神经系统受累少见(4%)。贫血72例(64%),白细胞增多92例(82%),中性粒细胞增多71例(63%),血小板增多53例(47%),ESR增快105例(94%),CRP升高92例(82%),ALT升高73例(65%),乳酸脱氢酶升高64例(57%),血清铁蛋白升高84%(59/70)。仅7%的患者用非甾体抗炎药可控制病情,其余均需要加用肾上腺皮质激素(激素),需再加用1种免疫抑制药占44%,需要加用2种免疫抑制药占13%。随访62例患者,获长期缓解56%,病情呈慢性持续性16%,反复发作23%,死亡3例。结论:AOSD是一种以发热、皮疹、关节痛、白细胞增多为主要特征的自身免疫性疾病,预后良好,多数患者用激素治疗有效,但对以关节炎表现为主以及合并内脏损害的患者应尽早使用免疫抑制药,以控制病情。

[ Objective： To investigate the clinical features, diagnosis, treatment and outcome of patients with adult onset Stillg disease （AOSD）. Methods： Clinical data of 112 patients with AOSD were analyzed. Results： Fever, arthritis, sore throat, rash and myalgia were the most common symptoms seen in AOSD. Respiratory system involvement was also common and present in 33% of the patients. Among them, 5 patients had interstitial lung disease. Cardiovascular system involvement was present in 17% , gastrointestinal system in 7% and nervous system in 4% of the patients. Anemia was present in 72 patients （64%）, leukocytosis in 92 （82%）, thrombocytosis in 53 （47%） and neutrophilia in 71 （63%） patients. An elevated erythrocyte sedimentation rate （ESR） in 105 （94%）, C-reactive protein （CRP） in 92 （82%）, increased liver enzymes in 73 （65%）, increased lactic dehydrogenase in 64 （57%）, and elevated serum ferritin levels in 59 （59/70, 84% ） patients were found. Seven percents of the patients were treated by non-steroid anti-inflammatory drug alone, while others needed corticosteroids. Forty-four percents of patients needed one additional immunosuppressive and 13% needed two. Sustained remission, chronic disease course and intermittence were seen in 57%, 16% and 23% of 62 patients, respectively, and 3 patients died. Conclusion： AOSD is an autoimmune disease of unknown etiology character- ized by fevers, rash, arthritis and leukocytosis. It is a relatively benign disease if diagnosed early and treated appropriately. Cortico- steroid is often effective, but immunosuppressive may be needed in patients with multiple arthritis and vital organ involvement for longterm remission.