Abstract
Fibrotic hypersensitivity pneumonitis is a complex interstitial lung disease that is not entirely understood. In its chronic and fibrotic form, hypersensitivity pneumonitis is one of the main mimickers of idiopathic pulmonary fibrosis (IPF). Distinguishing between these two conditions is challenging but is of particular clinical relevance. Two approved therapies are available for IPF, and a considerable number of clinical trials are now exploring newer pharmacological options. This impressive research effort is a consequence of new pathogenetic understanding, updated diagnostic criteria and a long history of pharmacological trials. Conversely, current knowledge gaps on pathogenesis of chronic hypersensitivity pneumonitis, coupled with lack of validated diagnostic criteria, make the management of this disease an unsolved clinical challenge. This also reflects the paucity of therapeutic clinical trials in this field. In this review, we describe the current evidence and the possible future options to approach this complex disease.
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Dr. Varone reports personal fees from Boehringer Ingelheim and Roche, outside the submitted work; Dr. Iovene reports personal fees from Boehringer Ingelheim and Menarini, outside the submitted work; Dr. Sgalla reports personal fees from Boehringer Ingelheim, outside the submitted work; Dr. Calvello reports personal fees from Boehringer Ingelheim, outside the submitted work; Prof. Richeldi reports personal fees from Sanofi-Aventis, personal fees from Roche, personal fees from ImmuneWorks, personal fees from Boehringer Ingelheim, personal fees from Celgene, personal fees from Nitto, personal fees from Fibrogen, personal fees from Promedior, personal fees from Bristol Myers Squibb, personal fees from DynaMed, outside the submitted work; Drs. Larici and Calabrese have nothing to disclose.
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Varone, F., Iovene, B., Sgalla, G. et al. Fibrotic Hypersensitivity Pneumonitis: Diagnosis and Management. Lung 198, 429–440 (2020). https://doi.org/10.1007/s00408-020-00360-3
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DOI: https://doi.org/10.1007/s00408-020-00360-3