• Francesco Varone, Bruno Iovene, Giacomo Sgalla, Mariarosaria Calvello, Angelo Calabrese, LariciAnna RitaARFondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.Università Cattolica del Sacro Cuore, Rome, Italy., and Luca Richeldi.
• Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
• Lung. 2020 Jun 1; 198 (3): 429-440.

AbstractFibrotic 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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