非特异性间质性肺炎不同病理类型的临床分析

目的比较不同病理类型的非特异性间质性肺炎(NSIP)的临床特点,以提高对NSIP的认识水平。方法用回顾性分析的方法对北京协和医院2001年2月至2005年8月期间收治的18例NSIP病例(经肺活检确诊为富细胞为主型和纤维化为主型)的临床资料进行总结分析。结果富细胞组5例均为女性,平均年龄(37±10)岁;纤维化组13例(男3例、女10例),平均年龄(46±13)岁。临床表现均以咳嗽、咳痰、呼吸困难、爆裂音为主要表现,杵状指少见;肺外表现以乏力和体重下降为多见。富细胞组和纤维化组抗核抗体阳性者分别为4例和5例,类风湿因子阳性者分别为0例和3例,两组患者均有轻度的红细胞沉降率增快、α1球蛋白升高。肺功能检查结果有限制性通气功能障碍和弥散功能障碍,动脉血气分析显示有轻度低氧血症,支气管肺泡灌洗液中淋巴细胞、中性粒细胞和嗜酸粒细胞有不同程度的升高,T淋巴细胞亚群分析提示以CD8+细胞为主。两组的胸部高分辨率CT以磨玻璃样影和双肺弥漫性间质纹理增厚为主要表现;部分患者出现胸膜增厚、纵隔淋巴结肿大的表现。转归富细胞组1例失访,其余4例病情好转;纤维化组1例在随诊8个月后死于肺部感染和呼吸衰竭,5例病情稳定,7例病情好转。结论NSIP富细胞为主型与纤维化为主型在临床表现及客观检查等方面无明显差别,但前者的女性患病率、抗核抗体阳性率稍高,预后更好;后者体重下降、爆裂音更为多见。两者的鉴别有赖于病理表现,对预后分析有一定提示意义。

Clinical analysis of different pathological patterns of nonspecific interstitial pneumonia

OBJECTIVE: To describe the clinical characteristics of 18 patients with different pathological patterns of nonspecific interstitial pneumonia (NSIP). METHOD: The clinical and radiological data of 18 patients with NSIP admitted into Peking Union Medical College Hospital from January 2001 to August 2005 were retrospectively analyzed. The diagnosis of NSIP was confirmed by surgical lung biopsy. RESULTS: Pathologically the cellular mainly pattern (CM-NSIP) was present in 5 patients, all of whom were female, and aged (37 +/- 10) years. The fibrosing mainly pattern (FM-NSIP) was found in 13 patients, 3 males and 10 females, with a mean age of (46 +/- 13) years. The main manifestations of respiratory system were cough, sputum production, dyspnea and inspiratory crackles. Clubbing fingers were found in only one patient with FM-NSIP. Fatigue and weight loss were the main extrapulmonary symptoms. The positive rate of antinuclear antibodies (ANA) of CM-NSIP and FM-NSIP patients were 4/5 and 5/13, respectively. The positive rate of rheumatoid factor of CM-NSIP and FM-NSIP patients were 0/5 and 3/13, respectively. In both groups, ESR and alpha1-globulin were slightly elevated. Pulmonary function tests showed restrictive ventilatory dysfunction and decrease of D(LCO), while arterial blood gas analysis showed mild hypoxemia. Increase of lymphocytes, neutrophils and eosinophils was demonstrated in bronchoalveolar lavage fluid, and the lymphocytes were predominantly CD8+-positive. Ground glass opacities and diffuse intralobular lines were the main manifestations of high resolution CT, while pleural thickening and mediastinal adenopathy were also found in some patients of both groups. Of the patients with CM-NSIP, one lost contact and the rest 4 showed clinical improvements during follow-up. In the FM-NSIP group, one patient died at 8 months after the initiation of therapy, 5 patients remained stable and 7 patients got clinical improvement. CONCLUSIONS: There are no significant differences between CM-NSIP and FM-NSIP in clinical manifestations, laboratory examinations and CT manifestations. But in this series CM-NSIP is found only in females, and with a higher positive rate of ANA and better outcomes. Weight loss and inspiratory crackles are more common in FM-NSIP. However, these two patterns of NSIP can only be differentiated by pathological examination, which is also valuable for prognosis.