Tomographic pattern and disease progress or mortality in familial pulmonary fibrosis

Abstract

Background: Familial pulmonary fibrosis (FPF) is defined as an idiopathic diffuse parenchymal lung disease affecting two or more members of the same primary biological family. Aim: to evaluete if the pattern of fibrosis on HRCT has a significant impact on disease progression or mortality in patients with FPF. Methods: Baseline clinical, functional and radiological data of a FPF population were retrospectively collected and analysed according to the 2018 IPF guidelines High Resolution Computerized Tomography (HRCT) classification. There was considered disease progress, decline in CVF more then 10% in a year. Results: 49 patients were included, 29 male and 20 female patients (age at diagnosis 53 ± 8.5 years-old), with a predominance of former smokers (51%), belonging to 31 families. Radiological analysis demonstrated the presence of a “UIP“ pattern at HRCT was 15 (29 %) of patients, “Probable-UIP” in 10 (19.5 %), “Indeterminate-UIP” in 7(13,5%) and “CT features most consistent with non-IPF diagnosis” in 17 (33%) . The main diagnostic was idiopathic pulmonary fibrosis (IPF), 21(41%). Initial forced vital capacity (FVC) was 75,07 ± 17,01 % of predicted and Rest SpO2 was 92 ± 5.8%. After analysis, the survival time 28.3 ± 17 months. No difference was observed in the survival or progression of the disease when we compared the “UIP“ pattern and “Probable-UIP” with “Indeterminate-UIP” and “CT features most consistent with non-IPF diagnosis”(p = 0,81) Reference: 1-Bennett D et al. Respiratory Medicine. 2017: 126: 75-83.