Antolini I, Miglioranzi P, Boner A L
Pediatr Med Chir. 1986 Sep-Oct;8(5):675-82.
Pulmonary fibrosis is a chronic disorder characterized by inflammation, injury and fibrosis of the alveolar structures and consequent loss of functional alveolar-capillary units. It involves a number of unresolved terminologic, pathogenetic, diagnostic and therapeutic controversies. The key to understanding the disease is the recognition that inflammation is the first event. The staging of the patients, important for correct therapy decision, is made using methods that specifically evaluate the inflammation such as open lung biopsy, bronchoalveolar lavage, and Gallium 67 scanning. The most rational therapeutic approach is to suppress the inflammation and to prevent further injury and scarring. The drugs of choice for treatment are corticosteroids (prednisone particularly). It is suggested to use large doses during the initial portion of the therapeutic trial followed by a tapering of the dose. If the patient does not respond to corticosteroids other immunosuppressive agents can be considered, particularly cyclophosphamide. The frequency and duration of response to these regimens remains to be defined.
肺纤维化是一种慢性疾病，其特征为肺泡结构的炎症、损伤和纤维化，以及随之而来的功能性肺泡-毛细血管单位丧失。它涉及许多尚未解决的术语、发病机制、诊断和治疗方面的争议。理解该疾病的关键在于认识到炎症是首要事件。对患者进行分期对于正确的治疗决策很重要，分期采用专门评估炎症的方法，如开胸肺活检、支气管肺泡灌洗和镓67扫描。最合理的治疗方法是抑制炎症并防止进一步的损伤和瘢痕形成。治疗的首选药物是皮质类固醇（特别是泼尼松）。建议在治疗试验的初始阶段使用大剂量，随后逐渐减少剂量。如果患者对皮质类固醇无反应，可以考虑使用其他免疫抑制剂，尤其是环磷酰胺。这些治疗方案的反应频率和持续时间仍有待确定。
