Servicio de Neumología, Hospital Recoletas Campo Grande, Valladolid, España.
Unidad de Enfermedades Pulmonares Intersticiales Difusas, Servicio de Neumología, Hospital Universitario de la Princesa, Universidad Autónoma de Madrid, Madrid, España.
Med Clin (Barc). 2022 Aug 26;159(4):189-194. doi: 10.1016/j.medcli.2022.02.020. Epub 2022 Jun 1.
Idiopathic pulmonary fibrosis is defined as a chronic progressive fibrosing interstitial pneumonia of unknown etiology. There are intrinsic and extrinsic risk factors that could favor the development of the disease in individuals with a genetic predisposition. The diagnosis is made by characteristic radiological and/or histological findings on high-resolution computed tomography and lung biopsy, respectively, in the absence of a specific identifiable cause. The median survival of the disease for patients without treatment is 3-5years from the onset of symptoms, although its natural history is variable and unpredictable. Currently, there are two antifibrotic drugs that reduce disease progression. The multidisciplinary approach will consider the nutritional and emotional status, physical conditioning, and treatment of comorbidities, as well as lung transplantation and palliative care in advanced stages. The following article reviews the fundamental aspects for the diagnosis and treatment of idiopathic pulmonary fibrosis.
特发性肺纤维化定义为一种原因不明的慢性进行性纤维性间质性肺炎。在具有遗传易感性的个体中，某些内在和外在的危险因素可能有利于疾病的发展。在没有特定可识别病因的情况下，该疾病的诊断依据是高分辨率计算机断层扫描和肺活检的特征性放射学和/或组织学表现。未经治疗的患者的中位生存时间从症状出现起为 3-5 年，尽管其自然病程是可变且不可预测的。目前，有两种抗纤维化药物可减缓疾病进展。多学科方法将考虑营养和情绪状态、身体状况、合并症的治疗以及晚期的肺移植和姑息治疗。以下文章回顾了特发性肺纤维化的诊断和治疗的基本方面。
