北京大学学报(医学版) ›› 2014, Vol. 46 ›› Issue (5): 805-808.

• 疑难/罕见病例分析 • 上一篇    下一篇

类风湿关节炎合并无肌病性皮肌炎伴多重肺损伤1例

刘爽*,安媛△,贾园,栗占国   

  1. (北京大学人民医院风湿免疫科,北京100044)
  • 出版日期:2014-10-18 发布日期:2014-10-18

A case of clinical overlap syndrome of rheumatoid arthritis and amyopathic dermatomyositis with multiple pulmonary injuries

LIU Shuang*,AN Yuan△,JIA Yuan,LI Zhan-guo   

  1. (Department of Rheumatology and Immunology,Peking University People’s Hospital,Beijing 100044,China)
  • Online:2014-10-18 Published:2014-10-18

摘要: 自身免疫疾病常导致肺部损伤,复习北京大学人民医院风湿免疫科收治的1例重叠综合征(类风湿关节炎合并无肌病性皮肌炎)伴多重肺部损伤的患者,结合文献探讨其病例特点及诊治经验。患者起病为类风湿关节炎,病程中出现以空洞为表现的肺部类风湿结节,排除结核、真菌等感染及肿瘤后,行病理检查确诊。此后又出现以发热为表现的机化性肺炎,予常规量激素治疗;机化性肺炎进展时出现典型手部Gottron征及技工手,无肌酶及肌电图改变,考虑为无肌病性皮肌炎,予激素冲击联合环磷酰胺治疗后机化性肺炎好转,但同时出现肺部感染,经暂停环磷酰胺、激素减量并联合抗感染治疗后感染好转。本例患者为重叠综合征,出现多种肺部损伤,如类风湿关节炎肺损害、免疫疾病合并的机化性肺炎、肺叶切除术后的支气管胸膜瘘、免疫抑制治疗过程中出现肺部真菌、细菌感染等,其诊疗经验可以加深对结缔组织病肺部损害的认识,并提高诊断和治疗水平。

关键词: 关节炎, 类风湿, 无肌病性皮肌炎, 肺损伤

Abstract: Autoimmune diseases can cause various kinds of lung injuries. Clinical features of a case of overlap syndrome with multiple pulmonary injuries were investigated, and the treatment experiences discussed. The patient suffered from rheumatoid arthritis (RA) at first, and then had a lobectomy surgery due to the rheumatoid nodules in her right lung. A year later her disease was diagnosed as amyopathic dermatomyositis (ADM) with typical Gottron’s sign, craftsmen hands and rapidprogressive organizing pneumonia (OP). After a combined treatment with glucocorticoids (GCs) and cyclophosphamide (CTX), her OP became better but lung infections progressed. Her lung infections eventually were cured after we used antibiotics and antifungal treatment while we ceased to use CTX and reduced the dosage of GCs. The clinical feature of the patient was overlap syndrome with a variety of lung injuries, such as pulmonary rheumatoid nodules, OP, secondary bronchopleural fistula and lung infections. Its diagnosis and treatment experiences could improve our understanding of pulmonary manifestations of connective tissue disease and improve our diagnosis and treatment level.

Key words: Arthritis rheumatoid, Amyopathic dermatomyositis, Lung injury

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