Schäfer Stephan C, Funke-Chambour Manuela, Berezowska Sabina
Institut für Pathologie im Medizin Campus Bodensee, Röntgenstraße 2, 88048, Friedrichshafen, Deutschland.
Institut für Pathologie, Uniklinik Köln, Köln, Deutschland.
Pathologe. 2020 Feb;41(1):46-51. doi: 10.1007/s00292-019-00747-x.
Idiopathic pulmonary fibrosis (IPF) plays a special role within the group of interstitial lung diseases (ILDs) due to its inexorable progression and its specific medical treatment. With a median survival of only 2-3 years from the time of diagnosis, the prognosis is worse than many carcinomas.In contrast to other ILDs, IPF does not respond to anti-inflammatory treatment with corticosteroids but rather demands a specific medical therapy. Even though this cannot cure the disease, it can prolong survival. Lung transplantation is the only cure for progressive lung fibrosis. The clinical course is individual and difficult to predict. Acute exacerbations accelerate the clinical course and lead to high mortality.The underlying pathomechanisms of IPF, with its complex immunological and inflammatory processes and external impacts, have been the focus of recent research. Lifestyle and environmental influences are held responsible for much of its natural history. Smoking, pneumotoxic medications, and inhalation of dusts are well-known risk factors. Likewise, genetic and hereditary factors play a crucial role.This short review focuses on the peculiarities of IPF within the group of ILDs, especially in relation to its underlying mechanisms and clinical progression.
特发性肺纤维化（IPF）在间质性肺疾病（ILDs）组中具有特殊地位，因其病情不可阻挡地进展以及特定的医学治疗方法。从诊断之时起，其平均生存期仅为2至3年，预后比许多癌症还要差。与其他ILDs不同，IPF对皮质类固醇抗炎治疗无反应，而是需要特定的医学疗法。尽管这种疗法无法治愈该疾病，但可以延长生存期。肺移植是进行性肺纤维化的唯一治愈方法。临床病程因人而异且难以预测。急性加重会加速临床病程并导致高死亡率。IPF的潜在发病机制，因其复杂的免疫和炎症过程以及外部影响，一直是近期研究的重点。生活方式和环境影响在很大程度上决定了其自然病程。吸烟、肺毒性药物和粉尘吸入是众所周知的危险因素。同样，遗传和遗传因素也起着关键作用。本简短综述重点关注IPF在ILDs组中的特殊性，特别是与其潜在机制和临床进展相关的方面。
