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成人斯蒂尔病:第一部分。法国65例患者的临床表现及并发症

Adult Still's disease: part I. Manifestations and complications in sixty-five cases in France.

作者信息

Masson C, Le Loët X, Lioté F, Renou P, Dubost J J, Boissier M C, Brithmer L, Brégeon C, Audran M

机构信息

Department of Rheumatology, Angers, France.

出版信息

Rev Rhum Engl Ed. 1995 Dec;62(11):748-57.

PMID:8869216
Abstract

DESIGN

a retrospective multicenter study conducted in France identified 65 cases of adult Still's disease. Data were recorded on a standardized questionnaire validated by the Inflammatory Joint Disease and Immunorheumatology Committee of the Société Française de Rhumatologie.

OBJECTIVES

(1) To compare clinical and laboratory findings in our patients with those reported in earlier studies, particularly two large series from Canada and Japan, respectively; (2) to describe the systemic and visceral complications associated with adult Still's disease.

RESULTS

as compared with the two above-mentioned series, our study group included more patients who had experienced onset of their disease after the age of 35 years and fewer patients with involvement of the liver, spleen, or lymph nodes. Rates of occurrence of arthritis, myalgia, sore throat, pleuritis, pericarditis, and abdominal pain were significantly higher in the Canadian series than in the other two series. Arthritis was absent in one fourth of our patients. Life-threatening complications included "Still's hepatopathy", disseminated intravascular coagulation (with hemophagocytosis in some cases), and "Still's myocarditis".

CONCLUSION

differences in the expression of adult Still's disease were found between patients from Canada, France, and Japan. Adult Still's disease can be responsible for life-threatening complications.

摘要

设计

在法国进行的一项回顾性多中心研究确定了65例成人斯蒂尔病病例。数据记录在由法国风湿病学会炎症性关节疾病和免疫风湿病委员会验证的标准化问卷上。

目的

(1)将我们患者的临床和实验室检查结果与早期研究报告的结果进行比较,特别是分别来自加拿大和日本的两个大型系列研究;(2)描述与成人斯蒂尔病相关的全身和内脏并发症。

结果

与上述两个系列相比,我们的研究组中35岁以后发病的患者更多,而肝脏、脾脏或淋巴结受累的患者更少。加拿大系列中关节炎、肌痛、咽痛、胸膜炎、心包炎和腹痛的发生率明显高于其他两个系列。我们四分之一的患者没有关节炎。危及生命的并发症包括“斯蒂尔肝病”、弥散性血管内凝血(某些病例伴有噬血细胞现象)和“斯蒂尔心肌炎”。

结论

在来自加拿大、法国和日本的患者之间发现了成人斯蒂尔病表现的差异。成人斯蒂尔病可导致危及生命的并发症。

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