Ioannis Organtzis, Evangelia Fouka, Christoforos Efthymiou, Violetta Mourouzidou, Georgios Spyropoulos, Theodoros Kontakiotis, Despina Papakosta
Background: Evaluation of characteristics and disease progression during twelve-month follow up in patients with idiopathic pulmonary fibrosis (IPF).
Methods: Retrospective study of patients with IPF followed on a regular basis in Interstitial Lung Diseases Clinic.
Results: Fifty-six patients were studied, 16 female and 40 male, with mean age 69.51±7.32 years (45–84) and mean disease duration since diagnosis 25.58±21.19 months. Seventeen patients demonstrated concomitant emphysema on HRCT. Pulmonary function tests were performed in 50 patients, which presented a mild restrictive pattern, with mean FEV1 =82.21±17.05%, FVC =74.66±17.30% and FEV1/FVC =84.39±7.68 and a moderately reduced transfer factor for carbon monoxide (TLCOSB), with a mean value of 45.79±14.60%. Patients were classified according the prognostic GAP index as class I (0–1) =4, II (2–3) =13, III (4–5) =28 and IV (>5) =5 patients. Pulmonary hypertension, with right ventricle systolic pressure (RSVP) >35 mmHg documented by cardiac ultrasound, was found in 12 from the 23 patients tested, with mean RVSP 44.50±11.49 mmHg. Thirty-seven patients underwent a six-minute walking test, with mean distance walked 345±148 m and mean SpO2 recorded in the beginning and after the end of the procedure 93±2.86% and 84±7.94%, respectively. Thirty-five patients were on antifibrotic therapy, with 30 patients receiving pirfenidone and 5 patients nintedanib, respectively, for a mean duration of 16.8±8.76 months. From the 37 patients that were followed for 12 months after diagnosis, 25 had been receiving pirfenidone and 2 patients were on nintedanib.
Conclusions: Fifty-nine percent of patients presented an increased GAP index at diagnosis, indicative of an unfavorable prognosis, a fact that might have been responsible for their functional deterioration. Our results emphasize the need for prompt initiation of treatment.
