A rare entity of interstitial lung disease, pleuropulmonary fibroelastosis: does it affect the chest wall geometry?

Abstract

Introduction Pleuropulmonary fibroelastosis (PPFE) is a rare type of interstitial lung disease (ILD); however, it may not be as rare as it was described. PPFE has been recognized increasingly worldwide during the past years. Patients and methods The study was held in the Chest Department, Kasr Al-Ainy hospitals, during the period from January 2015 till June 2018. Seventy patients were included and divided into two main groups. Group 1 included 36 cases with PPFE, diagnosed either radiologically alone or combined with histopathological examination of lung biopsy. Group 2 included 34 cases of hypersensitivity pneumonitis (HP) as controls. Group 1 was further subdivided into two subgroups: group A included patients with 19 PPFE without any other pattern of ILD, and group B included 17 cases of PPFE associated with other forms of ILD. Clinical assessment, BMI, and high-resolution computed tomography chest were done. The inner anteroposterior diameter (APD) and transverse diameter (TD) of the chest wall were measured, and the ratio between them was calculated. Results Significant female predominance was observed. Both groups of PPFE presented at earlier age than the HP group. Patients with PPFE had a lower body weight and BMI than HP group. There was a significant reduction in the APD and TD in both groups of PPFE than HP group. Conclusion Thirty-six cases with PPFE presented either alone or in association with other forms of ILD. Significant reduction in their chest wall APD in comparison with TD was observed, giving a characteristic flat shape of the chest. Further evaluation of this phenomena and its explanation is required.