Disease progression and survival in histology-proven hypersensitivity pneumonitis pattern: a retrospective cohort study

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Introduction: Hypersensitivity pneumonitis (HP) remains a diagnostic challenge despite recent advances in classification criteria. Histological confirmation is rarely performed but may improve diagnostic clarity. Aim: To investigate the diagnostic and prognostic value of a HP histological pattern. Methods: Retrospective study of all patients with HP pattern on lung biopsy at our institution (2012-2023), reviewed by expert pathologists. Outcomes included ILD progression (absolute decrease in FVC ⩾ 5%pred. or TLCO ⩾ 10%pred.) and mortality. Results: Among 49 patients included (surgical biopsies, n=39 (80%); transbronchial biopsies, n=10 (20%)), 57% were female aged 67±8 years-old at biopsy with a follow-up of 4.9±2.7 years. Histological HP pattern was definite in 36 (73%), mostly fibrotic (n=36, 73%) vs cellular (n=13, 27%) whereas the CT pattern was deemed typical in 14 cases (29%). Lung histology modified working diagnosis after multi-disciplinary discussion in 33 cases (67%), with HP (n=41, 84%) as most frequent diagnosis. During follow-up, ILD progression occurred in 35 patients (71%) (after a median of 2.9 years [1.2-4.8]) and 13 (27%) died. Smoking history increased risk for death (HR 6.41 95CI [1.34-30.6] p=0.02) whereas female sex was protective (HR 0.17 [0.04-0.66] p=0.01, univariate). All patients who died had experienced ILD progression vs 22 survivors (61%) (p=0.01). Lower TLCO and a fibrotic histological pattern were associated with ILD progression (HR 0.96 [0.93-0.99], p=0.02, HR 5.18 [1.68-16.0], p=0.004, respectively, multivariate). Conclusion: HP histology pattern has a significant impact on diagnostic reclassification and prognostic implications.

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