• Vasileios Kouranos, Joseph Jacob, Andrew Nicholson, and Elizabetta Renzoni.
• Interstitial Lung Disease Unit, Royal Brompton Hospital, National Heart and Lung Institute, Imperial College, Sydney Street, SW3 6NP London, UK. v.kouranos@rbht.nhs.uk.
• J Clin Med. 2017 Jun 15; 6 (6).

AbstractThe diagnosis of hypersensitivity pneumonitis (HP) relies on the clinical evaluation of a number of features, including a history of significant exposure to potentially causative antigens, physical examination, chest CT scan appearances, bronchoalveolar lavage lymphocytosis, and, in selected cases, histology. The presence of fibrosis is associated with higher morbidity and mortality. Differentiating fibrotic HP from the idiopathic interstitial pneumonias can be a challenge. Furthermore, even in the context of a clear diagnosis of fibrotic HP, the disease behaviour can parallel that of idiopathic pulmonary fibrosis in a subgroup, with inexorable progression despite treatment. We review the current knowledge on the diagnosis, management, and prognosis of HP with particular focus on the fibrotic phenotype.

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