Winterbauer R H, Hammar S P, Hallman K O, Hays J E, Pardee N E, Morgan E H, Allen J D, Moores K D, Bush W, Walker J H
Am J Med. 1978 Oct;65(4):661-72. doi: 10.1016/0002-9343(78)90855-0.
Twenty patients with diffuse interstitial pulmonary disease diagnosed by open lung biopsy received combined prednisone/azathioprine therapy. Twelve patients demonstrated improvement with therapy. Each patient's clinical presentation, roentgenologic features and pathologic findings were correlated with their therapeutic response. Patients with an illness of one year's duration or less had a more favorable response to therapy than patients with a greater than two year duration of illness. Patients with associated extrathoracic abnormalities (anemia, glomerulitis, hepatopathy) exhibited a better therapeutic response that those with only pulmonary disease. The biopsy material from each patient was quantitatively graded on 20 morphologic variables. Statistical analysis using multiple linear regression revealed that a single variable, degree of interstitial fibrosis, was more that 90 per cent accurate in separating those responsive to therapy from those who failed to respond. Patients who respond to treatment had less interstitial fibrosis. Neither the amount of alveolar septal inflammation nor intra-alveolar cellular reaction was discriminatory in predicting response to therapy. A beneficial response to therapy was reflected in both improved lung volumes and gas exchange. Eight patients appeared to have a selective beneficial effect from azathioprine.
20例经开胸肺活检确诊为弥漫性间质性肺疾病的患者接受了泼尼松/硫唑嘌呤联合治疗。12例患者治疗后病情改善。将每位患者的临床表现、放射学特征和病理结果与其治疗反应进行关联分析。病程在1年及以内的患者对治疗的反应比病程超过2年的患者更佳。伴有胸外异常（贫血、肾小球炎、肝病）的患者比仅患有肺部疾病的患者治疗反应更好。对每位患者的活检材料依据20个形态学变量进行定量分级。采用多元线性回归进行统计分析显示，单个变量——间质纤维化程度——在区分治疗反应者与无反应者方面的准确率超过90%。对治疗有反应的患者间质纤维化程度较轻。肺泡间隔炎症的程度和肺泡内细胞反应在预测治疗反应方面均无鉴别意义。治疗的有益反应体现在肺容积和气体交换的改善上。8例患者似乎从硫唑嘌呤治疗中获得了选择性的有益效果。
