Mullin Monica L, Churg Andrew, Ryerson Christopher J
Department of Medicine, University of British Columbia.
Department of Pathology, University of British Columbia, Vancouver, British Columbia, Canada.
Curr Opin Pulm Med. 2022 Sep 1;28(5):421-431. doi: 10.1097/MCP.0000000000000904. Epub 2022 Jul 22.
Recent guidelines have updated the classification of hypersensitivity pneumonitis, stratifying by the presence or absence of fibrosis as either fibrotic or nonfibrotic hypersensitivity pneumonitis. Fibrotic hypersensitivity pneumonitis represents up to 10% of interstitial lung disease in large cohort studies, and is occasionally even more common in some regions; however, there are many unknown aspects to the diagnosis and management. The goal of this review article is to summarize the management of fibrotic hypersensitivity pneumonitis.
Historically, the only treatment options for patients with hypersensitivity pneumonitis were antigen avoidance and corticosteroids, although other immunosuppressive therapies are increasingly endorsed by experts in the field. There is accumulating evidence that antifibrotic medications can be useful as a second-line therapy in some patients with fibrotic hypersensitivity pneumonitis who have progression despite immunosuppression. There remains no direct comparison of immunosuppressive vs. antifibrotic medication for the management of fibrotic hypersensitivity pneumonitis, but some clinical, radiological and pathological features may suggest greater likelihood of benefit from one option or the other.
We anticipate that future treatment of fibrotic hypersensitivity pneumonitis will consider a variety of patient features to suggest the most prominent underlying biology that will then be used to guide initial pharmacotherapy; however, additional data are still needed.
近期指南更新了超敏性肺炎的分类，根据是否存在纤维化将其分为纤维化型或非纤维化型超敏性肺炎。在大型队列研究中，纤维化型超敏性肺炎占间质性肺疾病的比例高达10%，在某些地区甚至更为常见；然而，其诊断和管理仍存在许多未知方面。本文综述的目的是总结纤维化型超敏性肺炎的管理。
从历史上看，超敏性肺炎患者唯一的治疗选择是避免接触抗原和使用皮质类固醇，尽管该领域的专家越来越认可其他免疫抑制疗法。越来越多的证据表明，抗纤维化药物对于一些尽管接受了免疫抑制治疗仍有病情进展的纤维化型超敏性肺炎患者可作为二线治疗药物。目前尚无关于免疫抑制药物与抗纤维化药物治疗纤维化型超敏性肺炎的直接比较，但一些临床、影像学和病理特征可能提示从一种或另一种治疗选择中获益的可能性更大。
我们预计，未来纤维化型超敏性肺炎的治疗将考虑多种患者特征，以提示最突出的潜在生物学机制，进而用于指导初始药物治疗；然而，仍需要更多数据。
