儿童结缔组织病相关性肺间质病变7例临床分析

Clinical analysis of 7 children with interstitial lung disease associated with connective tissue disease

目的提高对儿童结缔组织病(CTD)相关性肺间质病变(ILD)认识,以期早期诊断、早期治疗,提高疗效。方法分析7例儿童CTD(过敏性紫癜、幼年型特发性关节炎-全身型、系统性红斑狼疮和结节性脂膜炎-全身型)发生ILD的临床诊治经过,并进行相关文献复习。结果7例患儿肺高分辨CT(HRCT)均提示ILD,但相应呼吸道症状均不明显。其中4例有活动后气促,2例在肺HRCT提示ILD前始终有不明原因的心率增快,2例有氧饱和度下降,而7例患儿的胸片均无显著特异性改变。2例经大剂量甲基泼尼松龙及环磷酰胺(CTX)冲击治疗后临床症状迅速缓解,2～3周后复查肺HRCT示明显好转。结论儿童CTD发生ILD,常缺乏呼吸道表现,但于早期CTD原发病控制后仍存在不明原因的心率增快时,应警惕发生ILD可能。儿童CTD相关性ILD治疗以泼尼松或甲基泼尼松龙联合一种免疫抑制剂为佳。病情一旦呈现急性进展,应予大剂量甲基泼尼松龙及CTX冲击治疗,并可辅以大剂量丙球。对于急性进展的ILD应及时排除感染的可能。

Objectives To enhance the recognization of interstitial lung disease （ILD） associated with connective tissue disease （CTD） in children in order to diagnose early, treat timely, and improve therapeutical effect. Methods Clinical data of ILD associated with CTD （Henǒch-Schǒnlein purpura HSP, systemic lupus erythematosus SLE, juvenile idiopathic athritis So-JIA, Weber-Christian disease）were analyzed in 7 children admitted during Dec. 2005 to Apr. 2007, and relevant literatures were reviewed. Results ILD was pointed out by high resolution computer tomography （HRCT） in all 7 children although no obvious respiratory symptoms were presented in these cases. There were 4 cases with shortness of breath after activity, 2 cases with agnogenic increased heart rates, and other 2 cases with decreased PaO2, respectively. No special changes of chest X-ray were observed in all 7 cases with ILD. After the impulse therapy of high-dose methyl- prednisolone combinated with CTX, symptoms were quickly alleviated in 2 cases （with SLE or So-JIA）of 7 patinets with ILD, and their HRCT became normal or improved after 2 - 3 weeks. Conclusions It is usually no respiratory symptoms in children with ILD associated with CTD at early. The occurrence of ILD should be considered if there is agnogenic increased heart rates after the parimary diseases is controlled. HRCT is sensitive to ILD. The most common therapeutic approaches for ILD associated with CTD involves the use of oral steroids combination with immunosuppressive drugs （cy- clophosphamide, azathioprine and cyclosporin）. If ILD is presented with acute course, it will be feasible to use the impulse therapy of high-dose methylprednisolone combinated with CTX. High-dose intravenous injection of gamma globulin can also be given as an auxiliary therapy. However, infection must be excluded in children with acute ILD. （J C//n Peal/art,2007,25（9） ：759-762） Key words：