[Fibrotic hypersensitivity pneumonia: focus on pathology-relevant aspects of the new ATS/JRS/ALAT clinical guideline for the diagnosis of hypersensitivity pneumonia in adults]

Abstract

Hypersensitivity pneumonia (HP), also called exogenous allergic alveolitis, is a chronic interstitial pneumonia induced by a hypersensitivity reaction to an identified or unidentified antigen in exposed and susceptible individuals that may progress to terminal lung fibrosis. The diagnosis of HP presents a diagnostic challenge. Though therapeutically important, it may be particularly difficult to differentiate fibrotic HP, historically termed chronic HP, from idiopathic pulmonary fibrosis (IPF) or interstitial lung disease associated with connective tissue diseases (CTD-ILD). Multidisciplinary discussion and thus a synoptic evaluation of all findings is firmly established as the gold standard diagnostic approach in interstitial lung diseases including HP. Nonetheless, the high interobserver variability between experts from the individual disciplines (pulmonology, radiology, and pathology) and between experienced multidisciplinary teams in assessing the diagnostic probability of HP has highlighted the need for widely accepted guidelines.The present review summarizes pathology-relevant aspects of the new ATS/JRS/ALAT clinical practice guideline for the diagnosis of HP in adults.

Keywords: Chronic fibrotic lung disease; Exogenous allergic alveolitis; Granuloma; Hypersensitivity pneumonitis; Idiopathic interstitial Pneumonia.