Antifibrotic treatment response comparison of progressive pulmonary fibrosis and idiopathic pulmonary fibrosis

Abstract

Background/Aim: Idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) are two entities categorized fibrotic lung diseases. With a similar clinical presentation and treatment modalities in many cases, the line differentiating two diseases may not be evident. We aimed to evaluate the effectiveness of antifibrotic treatment and the course of fibrotic lung diseases. Material and Methods: The study included patients diagnosed with IPF and PPF who were given antifibrotic treatment and followed up for one year in our clinic. At one year, treatment response and radiological evaluation were investigated by high-resolution computed tomography. Results: Eighty-seven patients were included in the study (57 with IPF and 30 with PPF). Under antifibrotic treatment, there were no statistically significant losses in 6MWT, FVC, and DLCO values at the 6-month and 12-month evaluations. The most common side effects were photosensitivity for patients under the pirfenidone regimen, while diarrhea was predominantly observed in the PPF group. Radiological progression was observed in 22.9% of all patients at the yearly evaluation. Hospitalization requirements were more evident in the PPF group, with at least one hospitalization history present in 60% (n=18) of patients compared to 12.3% (n=7) of IPF patients. Conclusion: A personalized approach is preferred with similar clinical profiles for both treatment modalities, with specific side effects considered.