Keogh B A, Bernardo J, Hunninghake G W, Line B R, Price D L, Crystal R G
Am Rev Respir Dis. 1983 Jan;127(1):18-22. doi: 10.1164/arrd.1983.127.1.18.
Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal disorder in which chronic accumulation of neutrophils within the alveolar structures occurs. These cells with their large stores of preformed mediators likely play a major role in subsequent lung derangement. To evaluate the adjunctive use of intermittent high dose "pulse" corticosteroid therapy as a means of inhibiting neutrophil accumulation in the IPF lung, 5 patients were treated in a single blind random fashion with "high dose" corticosteroids (2 g methylprednisolone given intravenously once a week plus 0.25 mg/kg prednisone given orally daily) and 8 patients were treated with "low dose" corticosteroids only (0.25 mg/kg prednisone given orally daily). All patients had biopsy-proved disease in midcourse, and the 2 groups were matched for clinical and physiologic criteria. To evaluate the effect of these therapies on the quantity of neutrophils in the lungs of these patients, both groups underwent bronchoalveolar lavage and 67Ga scanning at the beginning and end of the 6-month study period; both methods gave an estimate of the intensity of the neutrophil alveolitis in these patients. Low dose corticosteroids had little effect on neutrophil accumulation (% neutrophils in lavage, - 5 +/- 8% change from baseline; 67Ga uptake, + 27 +/- 14% change from baseline), whereas high dose corticosteroids significantly reduced neutrophil accumulation (% neutrophils in lavage, - 46 +/- 8% change from baseline, p less than 0.02 compared with that in the low dose group; 67 Ga uptake, - 23 +/- 11% change from baseline, p less than 0.05 compared with that in the low dose group). In addition, 2 patients in the high dose group were reevaluated 6 months after cessation of the intermittent high dose pulse corticosteroids. Both had marked increases in lavage neutrophils compared with when they were receiving the high dose therapy (10 to 22% and 18 to 52%, respectively). These findings suggest that massive doses of intermittent intravenously administered corticosteroids may help to suppress the neutrophil component of the alveolitis of IPF.
特发性肺纤维化（IPF）是一种进行性的致命疾病，其特征为肺泡结构内嗜中性粒细胞的慢性积聚。这些细胞储存有大量预先形成的介质，可能在随后的肺部紊乱中起主要作用。为了评估间歇性高剂量“脉冲”皮质类固醇疗法作为抑制IPF肺部嗜中性粒细胞积聚的辅助手段的效果，5名患者以单盲随机方式接受“高剂量”皮质类固醇治疗（每周静脉注射1次2g甲基泼尼松龙加每日口服0.25mg/kg泼尼松），8名患者仅接受“低剂量”皮质类固醇治疗（每日口服0.25mg/kg泼尼松）。所有患者在病程中期均经活检证实患有该病，两组在临床和生理标准方面相匹配。为了评估这些疗法对这些患者肺部嗜中性粒细胞数量的影响，两组在6个月研究期开始和结束时均接受了支气管肺泡灌洗和镓67扫描；两种方法均对这些患者的嗜中性粒细胞肺泡炎强度进行了评估。低剂量皮质类固醇对嗜中性粒细胞积聚影响很小（灌洗中嗜中性粒细胞百分比，与基线相比变化为-5±8%；镓67摄取，与基线相比变化为+27±14%），而高剂量皮质类固醇显著减少了嗜中性粒细胞积聚（灌洗中嗜中性粒细胞百分比，与基线相比变化为-46±8%，与低剂量组相比p<0.02；镓67摄取，与基线相比变化为-23±11%，与低剂量组相比p<0.05）。此外，高剂量组的2名患者在间歇性高剂量脉冲皮质类固醇治疗停止6个月后进行了重新评估。与接受高剂量治疗时相比，两人灌洗中的嗜中性粒细胞均显著增加（分别从10%增至22%和从18%增至52%）。这些发现表明，大剂量间歇性静脉注射皮质类固醇可能有助于抑制IPF肺泡炎的嗜中性粒细胞成分。
