Alvarez-Sala R, Prados C, Garciá F R, Lázaro T C, García-Satúe J L, Pino J M, Villamor J
Servicio de Neumología, Hospital La Paz, Universidad Autónoma, Madrid.
An Med Interna. 1993 Jul;10(7):355-60.
Idiopathic pulmonary fibrosis (IPF) is a disease of unknown etiology affecting alveolo-interstitial structures. It is clinically characterized by progressive dyspnea and dry cough. In the physical exploration, crakles at the pulmonary bases and acropachies are usually detected. Its diagnosis has been a major clinical problem. Currently, a definitive diagnosis can be established with high resolution computerized axial tomography, bronchoalveolar lavage and open pulmonary biopsy. The next clinical problem is the assessment of activity, since it allows to differentiate subsidiary patients for receiving treatment. The description of new therapeutical alternatives has brought new perspectives in the management of these patients. Corticotherapy associated to immunosuppressors has allowed to reduce side effects and to obtain dramatic improvements. Lung transplantation, currently under study, will be the therapy of the future.
特发性肺纤维化（IPF）是一种病因不明的疾病，累及肺泡间质结构。其临床特征为进行性呼吸困难和干咳。体格检查时，通常可在肺底部闻及湿啰音并发现杵状指。其诊断一直是一个主要的临床问题。目前，通过高分辨率计算机断层扫描、支气管肺泡灌洗和开胸肺活检可明确诊断。下一个临床问题是评估疾病活动度，因为这有助于区分适合接受治疗的患者。新治疗方法的出现为这些患者的管理带来了新的视角。皮质激素联合免疫抑制剂治疗可减少副作用并取得显著疗效。目前正在研究的肺移植将成为未来的治疗方法。
