结缔组织病并间质性肺病90例的临床特点分析

Clinical manifestations of connective tissue diseases complicated with interstitial lung disease:an analysis of 90 cases

目的通过复习结缔组织病并间质性肺病(CTD-ILD)患者的临床表现、血气分析、肺功能检查等临床资料,以期提高对该组疾病的了解和认识。方法回顾性分析1997年1月-2007年12月北京世纪坛医院确诊的90例CTD-ILD患者的临床表现、血气分析和肺功能检查资料。结果CTD-ILD患者共90例,男39例,女51例,年龄11～87岁,平均年龄为(62±15)岁。其中类风湿性关节炎(RA)13例,系统性硬化症(SSc)2例,皮肌炎/多发性肌炎(DM/PM)9例,干燥综合征(SS)8例,系统性红斑狼疮(SLE)6例,重叠综合征(OS)9例,未定型结缔组织病(UD-CTD)10例,抗中性粒细胞胞质抗体相关性血管炎(AASV)33例。呼吸系统症状(如咳嗽、呼吸困难、咯血)的发生率高于全身症状(如发热、乏力、关节疼痛、皮肤损害等)。81例患者进行血气分析检查,其中低氧血症31例,Ⅰ型呼吸衰竭16例,Ⅱ型呼吸衰竭2例。45例患者进行肺功能检查,其中限制性通气功能障碍25例,阻塞性通气功能障碍2例,混合性通气功能障碍4例,弥散功能障碍39例。结论CTD-ILD患者呼吸系统症状的发生率高于全身症状,伴发肺间质病变时,结缔组织病的临床表现可不明显,因此在发现间质性肺病时应注意继发于结缔组织病的可能。CTD-ILD患者的血气分析主要表现为低氧血症和Ⅰ型呼吸衰竭,肺功能主要表现为限制性通气功能障碍和弥散功能障碍,合并阻塞性通气功能障碍时,应考虑有伴发细支气管炎的可能。

Objective To strengthen the understanding and knowledge of connective tissue diseases （CTD） complicated with interstitial lung disease （ILD） by reviewing the clinical manifestations, blood gas analysis, and pulmonary function data of patients. Methods The clinical manifestations, blood gas analysis, and pulmonary function data of 90 patients with CTD-ILD, who were treated from January 1997 to December 2007 in the in-patient department, were retrospectively analyzed. Results Thirty-nine patients were male and 51 were female, with an age range from 11 to 87 years old （average E62-1- 15~] years）. The CTD included 13 cases with rheumatoid arthritis （RA）, 2 with systemic sclerosis （SSc）, 9 with dermatomyositis （DM）/polymyositis （PM）, 8 with sjogren syndrome （SS）, 6 with systemic lupus erythematosus （SLE）, 9 with overlap syndrome （OS）, 10 with undifferentiated connectived tissue disease （UD-OTD）, and 33 with antineutrophil cytoplasmic antibody （ANCA） associated systemic vasculitis （AASV）. The incidences of respiratory symptoms, such as cough, dyspnea, and haemoptysis, were higher than those of general symptoms, such as fever, fatigue, arthralgia and skin lesion. Among the 81 cases who received blood gas analysis, 31 had hypoxemia, 16 had respiratory failure type Ⅰ , and 2 had respiratory failure type Ⅱ. Among 45 cases who received pulmonary function test, 25 had restrictive, 2 had obstructive and 4 had mixed ventilation defect. Reduced diffusing capacity （DLcodecreased） was observed in 39 cases. Conclusion Patients with CTD-ILD have higher incidences of respiratory symptoms and lower incidences of general symptoms. The clinical manifestations of CTD may not be prominent when there are interstitial lung diseases; therefore the possibility of ILD second to CTD should be considered in these patients. The characteristics of pulmonary function of patients with CTD-ILD are mainly restrictive ventilation defect and reduced diffusing capacity. When combined with obstructive ventilation defects, the possibility of bronchiolitis should be considered （Shanghai Med J, 2009, 32： 607-610）