235例成人斯蒂尔病的临床特点回顾性研究

Clinical characteristics of 235 patients with adult onset of Still's disease: A retrospective study

目的探讨成人斯蒂尔病(AOSD)的发病规律、临床特征及治疗。方法回顾性分析解放军总医院近15年住院的AOSD患者的临床资料。结果 1)235例患者中,平均发病年龄(34.1±12.9)岁,16-35岁发病占54.5%;男∶女为1.0∶2.3。所有患者均有发热,皮疹90.6%,关节痛86.0%。2)白细胞(WBC)升高者占67.0%,中性粒细胞≥80%占63.9%,血沉(ESR)、C-反应蛋白(CRP)、血清铁蛋白(SF)升高者分别为95.7%、93.0%及57.1%,近50%患者肝功能异常,>90%抗核抗体(ANA)和类风湿因子(RF)阴性。3)73.2%的患者应用激素联合免疫抑制剂治疗;应用激素未加免疫抑制剂的占17.9%。4)应用大剂量激素治疗后白细胞较治疗前升高,20d内降至正常的10%左右,20d后降至正常的约50%,明显增多。结论 AOSD发病以青壮年为主,女性多于男性;发热、皮疹、关节痛等特征性的临床表现及WBC、SF等化验指标升高是诊断本病的关键。一般需激素治疗,但对病情不易控制或反复发作的需联合免疫抑制剂;应用大剂量激素治疗>20d WBC才会逐渐降至正常。

Objective To study the onset rule,clinical characteristics and treatment of adult onset Still＇s disease（AOSD）.Methods Clinical data about 235 AOSD patients admitted to our hospital in the past 15 years were retrospectively analyzed.Results AOSD occurred in 235 patients at the age of 34.1±12.9 years,and in 54.5% of the patients at the age of 16-35 years,with a female/male ratio of 1.0∶2.3.Fever occurred in all the patients with rash and arthralgia appeared in 90.6% and 86.0% of the patients,respectively.The number of leucocytes was increased in 67.0% of the patients with neutrophils≥80% in 63.9% of the patients.The erythrocyte sedimentation rate（ESR）,C-reactive protein（CRP） and serum ferritin（SF） levels were elevated in 95.7%,93.0% and 57.1% of the patients,respectively.The liver function was abnormal in almost 50% of the patients.The antinuclear antibody（ANA） and rheumatoid factor（RF） were negative in over 90% of the patients.Combined Glucocorticosteroids（GC） plus immunosuppressive agents were used in treatment of 73.2% of the patients with only GC used in treatment of 17.9% of the patients.The number of leukocytes was increased in the patients after treatment with a high dose of GC,which was then decreased to normal in about 10% of the patients within 20 days of treatment and in about 50% of the patients after 20 days of treatment.Conclusion AOSD occurs mainly in young or middle-aged individuals and more in females than in males.Its clinical manifestations include fever,rash and arthralgia.Laboratory findings,such a higher number of WBC and an elevated SF level,are the key points for its diagnosis.AOSD patients are usually treated with GC,but those with their AOSD not easily controlled or frequently relapsed should be treated with combined GC and immunosuppressive agents.The number of leukocytes is gradually decreased to normal after 20 days of treatment with a high dose of GC.