摘要目的 探讨糖皮质激素治疗特发性非特异性间质性肺炎(INSIP)的疗效及预后.方法 选择经临床、影像学和病理学(CRP)诊断的INSIP患者29例,根据有无纤维化分为INSIP-1组(富细胞型,9例)和INSIP-2组(混合型和纤维化型,20例),经CRP诊断的30例普通型间质性肺炎(UIP)患者作为对照组(UIP组),回顾性分析3组患者糖皮质激素治疗的疗效及随访结果,用Kaplan-Meier法估计生存曲线.结果 INSIP-1组的发病中位年龄(48.0岁)低于INSIP-2组(56.0岁)和UIP组(58.5岁),INSIP-1组的病程[(60±28)个月]长于INSIP-2组[(48±33)个月]和UIP组[(44±23)个月],但差异无统计学意义(F=1.22,P＞0.05);INSIP-1组糖皮质激素有效例数(9/9)明显高于INSIP-2组(11/20)和UIP组(2/30),差异均有统计学意义(均P＜0.05);INSIP-1组随访时间[(56±27)个月]明显长于INSIP-2组[(23±18)个月]和UIP组[(25±17)个月],显效例数(6/9)明显高于INSIP-2组(9/20)和UIP组(0/30),差异均有统计学意义(F=9.224,均P＜0.05),死亡例数(0/9)明显低于INSIP-2组(4/20)和UIP组(16/30),差异有统计学意义(均P＜0.05).结论 INSIP纤维化程度与糖皮质激素疗效和预后密切相关,糖皮质激素治疗富细胞型INSIP有较好的反应和预后,纤维化型INSIP的预后较差,明确诊断和分型具有重要的临床意义.

abstractsObjective To investigate the efficacy of glucocorticoid therapy in idiopathic nonspecific interstitial pneumonia ( INSIP). Methods Twenty-nine cases of INSIP confirmed by clinical- radiological- pathological (CRP) diagnosis were collected and classified into 2 groups according to the degree of fibrosis; INSIP-1 (including 9 cases of cellular type) and INSIP-2 (20 cases of mixed and fibrotic type). Thirty cases of usual interstitial pneumonia (UIP) confirmed by CRP diagnosis served as the control. Clinical and pathological features, therapeutic effects of glucocorticoids and the follow-up results were retrospectively analyzed and the survival curves were evaluated by Kaplan-Meier method. Results The mean age at onset of INSIP-1 group [ (48 ±5) years] was significantly younger than INSIP-2 group [ (52 ±11) years] and the UIP group [ (57 ± 14) years]. The course of disease in INSIP-1 group [ (60 ±28) months] was longer than that in INSIP-2 group [(48 ±33) months] and that in the UIP group [(44 ±23) months], but the differences were not statistically significant (F = 1. 22, all P > 0. 05 ). The efficacy rate of glucocorticoid treatment in INSIP-1 group (9/9) was higher than that in INSIP-2 group (11/20) and that in the UIP group (2/30), the differences being statistically significant (all P < 0.05). The follow-up period for INSIP-1 group [ (56 ±27) months] was significantly longer than for INSIP-2 group [ (23 ±18) months] and for the UIP group [ (25 ± 17) months] , and the rate of significant improvement (6/9) was higher than that of the INSIP-2 group (9/20) and the UIP group (0/30) , the differences being statistically significant (F = 9. 224, all P <0. 05). The mortality of INSIP-1 group (0/9) was lower than that in INSIP-2 group (4/20) and the UIP group (16/30), the difference being statistically significant (exact propability value 0. 000 - 0. 005, P < 0. 05). Conclusions The degree of fibrosis of INSIP is closely correlated with the effect of glucocorticoid therapy and progonosis. The cellular type has a favorable reaction to glucocorticoid therapy and a better prognosis as compared to the fibrotic type.