Abstract：
Idiopathic pulmonary fibrosis (IPF) is a severe progressive fibrotic disease of the lung. Its etiology is not yet completely clear. The affected population is exclusively older than 40 years with maximum incidence in the age categories of 60 to 70 years. Its worldwide prevalence varies from 2 to 29/100 000 people (in the Czech Republic 5-6/100 000). Annual incidence is constantly rising, mainly thanks to the ever-improving diagnostic possibilities. Untreated IPF disease causes rapid structural and functional devastation of the lungs with development of respiratory insufficiency and death of the patient within 2 to 3 years after diagnosis, prognosis with IPF is therefore fully (without any exaggeration) comparable to untreated bronchogenic carcinoma. In recent years, the prognosis and quality of life of patients have significantly improved thanks to available specific antifibrotic treatment, which can substantially slow down the disease progression and thus prolong survival. However, a necessary condition for the timely treatment initiation is a quick and accurate diagnosis. The following case report describes a protracted journey to the correct diagnosis in a patient with atypical radiological findings, so that the definitive diagnosis was established only as a result of a surgical lung biopsy.
摘要：
特发性肺纤维化（IPF）是一种严重的进行性肺纤维化疾病。其病因尚未完全明确。受影响的人口年龄完全在40岁以上，60至70岁年龄段的发病率最高。其全球患病率从2到29/100000人（在捷克共和国5-6/100000）不等。年发病率在不断上升,主要归功于不断改进的诊断可能性。未经治疗的IPF疾病会导致肺部结构和功能迅速破坏，并在诊断后2至3年内出现呼吸功能不全和死亡。因此，IPF的预后与未经治疗的支气管癌完全相当（毫不夸张）。近年来,患者的预后和生活质量有显著改善得益于可用的特异性抗纤维化治疗，这可以大大减缓疾病的进展，从而延长生存期。然而,及时开始治疗的必要条件是快速准确的诊断。以下病例报告描述了对具有非典型放射学发现的患者进行正确诊断的漫长旅程，因此，只有通过外科肺活检才能确定明确的诊断。
