Clinical Features and Prognosis of Adult-onset Still's Disease: 61 Cases from China

Clinical Features and Prognosis of Adult-onset Still's Disease: 61 Cases from China
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中国成人斯蒂尔病 61 例临床特征及预后

DOI:
10.3899/jrheum.080365
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发表时间:
2009-05-01
影响因子:
3.9
通讯作者:
Yang, Cheng-De
Yang, Cheng-De
中科院分区:
医学2区
文献类型:
--
作者:
Zeng, Ting;Zou, Yu-Qiong;Yang, Cheng-De

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目标。目的探讨成人斯蒂尔病(AOSD)的发病特点、临床特点、预后因素及治疗方法。对61例AOSD患者的临床资料进行回顾性分析。常见临床表现为发热(100.0%)、皮疹(88.5%)和关节炎(82.0%)。实验室检查结果如下:白细胞增多(83.6%),血沉升高(100.0%),转氨酶升高(23.0%),铁蛋白升高(79.6%),抗核抗体阴性(88.5%),类风湿因子阴性(88.5%)。61例患者中,44.3%表现为单循环病态,29.5%至少复发一次,16.4%表现为慢性关节病程,9.8%死亡,多死于肺部感染和呼吸衰竭。根据病程将61例患者分为两组:预后良好组(周期性病程45例)和预后不良组(慢性病病程或死亡16例)。我们分析了两组的预后因素,发现胸膜炎、间质性肺炎、铁蛋白水平升高以及强的松龙1 mg/kg/d用药3天后发热仍未消退是对患者不利的预后因素。AOSD。结论:AOSD。AOSD患者具有复杂的症状,没有具体的实验室结果。我们的结果表明,AOSD不是一种相对良性的疾病,特别是在对大剂量泼尼松无效的病例中。(第一次发布于2009年3月1日;风湿病杂志2009年:36:1026-31;DOI:10.3899/jrangum.080365)
Objective. To describe the onset, clinical features, prognostic factors, and treatment of adult-onset Still's disease (AOSD) in cases from China.Methods. Sixty-one Chinese patients with AOSD were analyzed retrospectively.Results. Common clinical features were fever (100.0%), rash (88.5%), and arthritis (82.0%). The laboratory findings were as follows: leukocytosis (83.6%), increased erythrocyte sedimentation rate (100.0%), elevated transaminase concentrations (23.0%), elevated ferritin levels (79.6%), negative antinuclear antibody (88.5%), and negative rheumatoid factor (88.5%). Of the 61 patients, 44.3% exhibited a monocyclic disease pattern, 29.5% experienced disease relapse at least once, 16.4% exhibited chronic articular course, and 9.8% died; most deaths were due to pulmonary infection and respiratory failure. Based on the disease course, we divided the 61 patients into 2 groups: those with favorable outcome (cyclic disease course, n = 45) and unfavorable outcome (chronic disease course or death, n = 16). We analyzed the prognostic factors for the 2 groups, and found that pleuritis, interstitial pneumonia, elevated ferritin levels, and failure of fever to subside after 3 days of prednisolone at 1 mg/kg/day were unfavorable prognostic factors for patients. with AOSD.Conclusion. Patients with AOSD had complex symptoms with no specific laboratory findings. Our results indicate that AOSD is not a relatively benign disease, especially in cases that are refractory to high doses of prednisone. (First Release March 1 2009; J Rheumatol 2009:36:1026-31; doi: 10.3899/jrheum.080365)