Brereton Christopher J, Jo Helen E
Dept of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, Australia.
Clinical and Experimental Sciences, University of Southampton, Southampton, UK.
Breathe (Sheff). 2020 Sep;16(3):200086. doi: 10.1183/20734735.0086-2020.
An acute exacerbation of idiopathic pulmonary fibrosis (AEIPF) is a potentially fatal complication of an already debilitating disease. Management is currently centred on delivering excellent supportive care and identifying reversible triggers. Despite growing international awareness and collaboration, no effective therapies have been identified. Corticosteroids are often the mainstay of treatment; however, the evidence base for their use is poor. Here, we review our current understanding of the disease process and how to manage it, with a focus on the role of corticosteroid therapy.
特发性肺纤维化急性加重（AEIPF）是一种本就使人虚弱的疾病的潜在致命并发症。目前的治疗重点是提供优质的支持性护理并识别可逆性诱因。尽管国际上对此的认识和合作不断增加，但尚未确定有效的治疗方法。皮质类固醇通常是治疗的主要手段；然而，其使用的证据基础薄弱。在此，我们回顾目前对该疾病过程的理解以及如何进行管理，重点关注皮质类固醇治疗的作用。
