北京大学学报(医学版)

• 病例报告 • 上一篇    

皮肌炎继发干燥综合征伴肺间质病变的血清人Ⅱ型肺泡细胞表面抗原变化1例

余建峰1,2*,金月波1*,何菁1△,安媛1, 栗占国1△   

  1. (1. 北京大学人民医院风湿免疫科,北京100044; 2. 湖北省第三人民医院肾病风湿科,武汉430000)
  • 通讯作者: 何菁,栗占国 E-mail: hejing1105@126.com, zgli99@aliyun.com

Changes of serum Krebs von den Lungen-6 levels in interstitial lung disease associated with dermatomyositis and secondary Sj-gren’s syndrome: a case report

YU Jian-feng1,2*, JIN Yue-bo1*, HE Jing1△, AN Yuan1, LI Zhan-guo1△   

  1. (1. Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing 100044, China; 2. Department of Nephrology and Rheumatology, The Third People’s Hospital in Hubei Province, Wuhan 430000, China)
  • Contact: HE Jing,LI Zhan-guo E-mail: hejing1105@126.com, zgli99@aliyun.com

摘要: 自身免疫性疾病往往可继发多脏器多系统损害,肺是常见受累器官之一,而自身免疫性疾病导致的肺间质病变(interstitial lung diseases, ILDs),往往以肺间质和肺泡腔不同形式和程度的炎症及纤维化为主要病理改变,病情进展迅速,最终结局为呼吸衰竭。常见的与ILDs相关的自身免疫性疾病有系统性硬化症、类风湿关节炎、系统性红斑狼疮、原发性干燥综合征、多发性肌炎/皮肌炎等。文献报道,一种高分子量糖蛋白——人Ⅱ型肺泡细胞表面抗原(Krebs von den Lungen6,KL-6)与上述疾病所致的ILDs关系密切。KL-6在正常肺组织的Ⅱ型肺泡细胞表达,当发生ILDs时,Ⅱ型肺泡细胞再生,KL-6明显升高,不仅出现于ILDs患者的肺泡灌洗液中[1],同时也存在于血清中[2]。现报道北京大学人民医院风湿免疫科的1例自身免疫性疾病合并ILDs的患者治疗前后血清KL6的变化情况,并结合临床和影像学特点来评估病情,指导治疗。

关键词:  皮肌炎, 干燥综合征, 肺间质病变, 血清人Ⅱ型肺泡细胞表面抗原

Abstract: Interstitial lung diseases (ILDs) are a diverse group of pulmonary disorders characterized by various patterns of inflammation and fibrosis in the interstitium of the lung. The underlying pathogenesis of ILDs is complex and associated with multiple rheumatologic conditions, such as systemic sclerosis, rheumatoid arthritis, pollymyositis and dermatomyositis, Sj-gren’s syndrome, and systemic lupus erythematosus. As the disease progresses, excessive pulmonary fibrosis impairs alveolar gas exchange and damages pulmonary function. The common methods to diagnose ILDs, such as clinical manifestations, pulmonary function test, and radiological examinations are not specific for ILDs and not able to diagnose ILDs at the early stage due to their low sensitivity. So, the easy way is important to diagnose ILDs. One important biomarker for ILDs is the high-molecular-weight glycoprotein, Krebs von den Lungen-6(KL-6). KL-6 encoded by the MUC1 gene is a mucin-like glycoprotein with high molecular weight and expressed predominantly on the cell surface of type Ⅱ alveolar epithelial cells, and  is detectable in the serum of patients with ILDs. We here report a case of ILDs associated with dermatomyositis and secondary Sj-gren’s syndrome. A 60-year-old woman was admitted to our hospital with the chief complaints of debilitation, dry mouth, dyspnea and astasia. ILDs associated with dermatomyositis and secondary Sj-gren’s syndrome was diagnosed clinically when the following criteria were satisfied: (1) development of dyspnea within 2 months of presentation, (2) pulmonary dispersion dysfunction, (3) bilateral infiltrative shadows on chest high resolution computed tomography (HRCT). She was treated with prednisone 50 mg/d prior to admission, but the result of therapy was not good. In our hospital she was treated with intravenous methylprednisolone and cyclophosphamide and oral hydroxychloroquine sulfate. Subsequently, her serum KL-6 levels gradually decreased after treatment, pulmonary diffuse function improved, and the improvement in the clinical manifestation and HRCT findings were observed. Nevertheless, the combination treatment of glucocorticoid and cyclophosphamide had contributed to the favourable outcomes. In conclusion, detection of serum KL6 levels in ILDs associated with connective tissue diseases may be be-neficial to making a definitive diagnosis, predicting the prognosis and monitoring the disease activity, which would be of great help in clinical practice. However, a well-designed clinical study with more patients and a longer follow-up period are required to arrive at a more conclusive judgment on the role of serum KL-6 in patients with ILDs.

Key words: Dermatomyositis, Sj-gren’s syndrome, Interstitial lung disease, Serum Krebs von den Lungen-6

中图分类号: 

  • R593.2
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