73例成人斯蒂尔病临床分析

Clinical Analysis of Seventy-Three Patients with Adult Onset Still's Disease

目的了解成人斯蒂尔病(AOSD)的病情特点及疾病转归。方法分析了73例AOSD患者临床及长期随访资料。结果 AOSD临床以发热、皮疹、关节痛/关节炎为主要表现,白细胞升高、骨髓培养(-)、血沉、C反应蛋白及血清铁蛋白升高、抗核抗体(-)是本病的实验室特征。发病后患者均应用肾上腺糖皮质激素,部分患者加用缓解病情抗风湿药。治疗后28例(38.4%)病情长期稳定,其中13例停用药物治疗;36例(49.3%)病情反复发作,其中24例(66.6%)1年内反复发作,8例(22.2%)3年内间歇发作;2例(2.7%)转变为系统性红斑狼疮;4例(5.5%)演变成类风湿关节炎;2例为未定性关节炎;1例死亡。结论 AOSD临床表现复杂多样,病情演变走向呈多种模式,规则用药是保持病情稳定的关键。

Objective To investigate the clinical characteristics and outcome of adult onset Still＇s disease.Methods A retrospective study was used to analyze the clinical data of seventy-three patients with adult onset Still＇s disease and a prospective study was carried out to analyze clinical outcome of these patients.Results Fever,rash,and arthralgia/arthritis were the main performance in AOSD＇s patients.Leucocytosis,bone marrow culture（-）,erythrocyte sedimentation rate,c-reactive protein and serum ferritin rise,and antinuclear antibodies（-） were AOSD＇s laboratory characteristics.After the outbreak of the disease,the patients were all applied in adrenal Glucocorticoids,some patients were combined with disease-modifying anti-rheumatic drugs（DMARDS）.28 patients were in stable condition after treatment,which amounts to 38.4%,thirteen cases of these patients needed no medicine;the disease attacked recurrently in 36 patients,which amounted to 49.3%,twenty-four cases of these thirty-six patients（66.6%） were attacked recurrently in one year,and eight cases of these thirty-six patients were attacked intermittently in three years;two cases of these eight patients converted to systemic lupus erythematosus;four cases converted to rheumatoid arthritis and the other two cases converted to undefined arthritis;1 patient died.Conclusion Clinical manifestation of adult onset Still＇s disease was complex and clinical outcome converted to several different outcome,regulated medication was the key point to ensure stability of the disease.