Coexistence of pleuroparenchymal fibroelastosis and hypersensitivity pneumonitis – analysis of prognostic impact

Abstract

<b>Introduction:</b> Pleuroparenchymal fibroelastosis (PPFE) may coexist with other interstitial lung disease (ILD), such as hypersensitivity pneumonitis (HP), but its prognostic significance is uncertain. <b>Aim:</b> To assess the prevalence of radiologic PPFE-like findings in a cohort of HP patients and to evaluate its clinical significance and prognostic impact. <b>Methods:</b> Retrospective analysis of chest HRCT of 250 HP patients. Radiological presence of PPFE was defined as definite, consistent or inconsistent (Reddy T.L. <i>et al</i>. Eur Respir J 2012;40: 377–385). Functional and clinical outcomes were compared with a random control HP group without PPFE (1:2 ratio). <b>Results:</b> Findings of definite or consistent with PPFE were present in 17 (6.8%) of the HP cohort. When compared to 34 HP controls, patients with PPFE had lower baseline DLCO values (47.3 ±15.2 vs 60.6 ±18.9 % of predicted, p=0.020). Although worst FVC values in the PPFE group, differences were not significant (85.7 ±20.3 vs 91.1 ±22.3 % of predicted, p=0.414). Concerning disease progression in a 2 year period, defined as decline for absolute values ≥10% for FVC or ≥15% for DLCO, no differences were found (X2 (1)= 0.046, p=0.831). Regarding other prognostic criteria, there were no differences among groups, such as long-term oxygen therapy need (p=0.503), number of exacerbations (p=0.991) or mortality (p=0.728). <b>Conclusions:</b> Coexistence of PPFE in HP was related to impaired diffusion lung capacity at diagnosis, but we found no impact in the prognosis in this Portuguese sample. A larger sample size is needed to clarify these potential associations.