雷诺现象为首发表现的自身免疫性疾病疾病谱及临床特点

Subset of Disease and Clinical Features of Autoimmune Disease with the Initial Presentation of Raynaud's Phenomenon

目的观察雷诺现象为首发临床表现的自身免疫性疾病疾病谱,以及其临床受累脏器的特点。方法选择2012年10月至2013年3月北京协和医院风湿免疫科门诊以雷诺现象为首发临床表现的自身免疫性疾病患者。除实验室常规检查外,所有患者均行胸部高分辨CT及超声心动图检查。结果共135例患者纳入研究,男9例,女126例,以系统性红斑狼疮(45.9%)、系统性硬化症(20.7%)及未分化结缔组织病(15.6%)最常见。系统受累以皮肤黏膜(97例,71.9%)、关节(86例,63.7%)及肺部受累(72例,53.3%)多见。其中合并肺间质病变者63例(46.7%),以系统性红斑狼疮(29例,46.8%)、系统性硬化症(17例,60.0%)及混合性结缔组织病(6例,83.3%)多见;合并肺动脉高压者37例(27.4%),以系统性红斑狼疮(16例,25.8%)、系统性硬化症(10例,35.7%)、未分化结缔组织病(4例,19.7%)及原发性干燥综合征(2例,28.6%)多见。结论雷诺现象可以是多种自身免疫性疾病的早期表现,尤其是系统性红斑狼疮及系统性硬化症。合并雷诺现象的患者更易出现肺部受累,尤其是肺间质病变及肺动脉高压,应重视在发病初期进行筛查。胸部高分辨CT及超声心动图检查分别是早期筛查肺间质病变及肺动脉高压的简便有效的方法。

To observe the subset of disease and clinical features of patients with Raynaud＇ s phenomenon as the initial presentation. Methods One hundred and thirty-five outpatients with Raynaud＇ s phenomenon as the initial presentation who were diagnosed with connective tissue disease in the department of rheumatology of Peking Union Medical College Hospital from October 2012 to March 2013 were enrolled. Chest high-resolution computer tomography （HRCT） and ultrasonogram （UCG） examination were performed in all cases. Results There were 9 males and 126 females. Systemic lupus erythematosus （SLE） （45.9%）, systemic sclerosis （SSc） （20.7%） and undifferentiated connective tissue disease （UCTD） （ 15.6% ） were the common final diagnosis of these patients. Systems involved in these patients were skin and mucous membrane （97 cases, 71.9% ）, joint （86 cases, 63.7% ）, lung involvement （72 cases, 53.3% ）. Sixty-three cases had interstitial lung disease, including patients with SLE （29 cases,46.8% ）, SSc （ 17cases, 60.0% ） and mixed connective tissue disease （6 cases, 83.3% ）, etc. Thirty- seven cases had PAH, including patients with SLE （ 16cases, 25.8% ） , SSc （ 10 cases, 35.7% ） , undifferentiated connective tissue disease（4 cases, 19.7% ）and primary Sjogren＇ s syndrome （2 cases, 28.6% ）. Conclusion Raynaud＇ s phenomenon can be an early manifestation in a variety of autoimmune diseases, especially in SLE and SSc. Patients with Raynaud＇ s phenomenon are more likely to have pulmonary involvement, especially ILD or/and PAH, therefore, more attention should be paid to these patients. Chest HRCT and UCG are simple and effective methods for early screening of interstitial lung disease and pulmonary arterial hypertension earlier.