Familial pulmonary fibrosis as aggressive as sporadic IPF

Patients with familial pulmonary fibrosis (FPF) share an equally aggressive disease progression as those with sporadic idiopathic pulmonary fibrosis (IPF) even though they are younger and less exposed to tobacco, reveals a study.

A total of 618 patients were included in this analysis, of whom 76 (12 percent) met the criteria for FPF. Patients with FPF were significantly younger than those with sporadic IPF (median age, 65 vs 72 years; p=0.0001).

There was no significant between-group difference in terms of male gender proportion and smoking status. However, the number of pack-year among current and former smokers was lower in those with FPF (20 vs 25; p=0.02). Additionally, antifibrotic treatment was given to 87 percent of FPF patients and 76 percent of sporadic IPF patients (p=0.047).

FPF and sporadic IPF patients showed similar baseline pulmonary function tests, median time before progression, and transplant-free survival. Notably, the genetic testing done in a subgroup of patients identified 10 telomerase-related gene variants.

“These results warrant early referral of FPF patients to expert centres for optimal management,” the authors said.

This multicentric prospective longitudinal and observational study (the Belgian and Luxembourg IPF registry; PROOF-NEXT) was carried out in Belgium and Luxembourg. The authors compared the characteristics and clinical course of patients with suspected FPF and sporadic IPF.

Suspected familial fibrosis was defined as “the occurrence of the disease in a patient younger than 50 and/or having at least one relative affected by pulmonary fibrosis.”