Clinical Experience with Pirfenidone in Connective Tissue Diseases Related Interstitial Lung Diseases

Objective: Pirfenidone is a new, anti-fibrotic drug used for the treatmentof idiopathic pulmonary fibrosis (IPF). This study was designed to verify the effect of pirfenidone administrated in connective tissue diseases related interstitial lung disease (CTD-ILD). Methods: Twenty two patients diagnosed with CTD-ILD were administrated with a 6-months treatment with or without pirfenidone. At baseline and at months 3rd and 6th, pulmonary function tests, six minute walk distance (6MWD) and HRCT scores were performed. Results: PFD group presented a significant improvement in TLC in the 3rd and 6th month points and DLco was greatly increased in 6th month point, which were all adjusted by the baseline levels, compared with control group (p=0.043, 0.048 and 0.043, respectively). During the follow-up period, TLC was also improved compared to the initial time point and DLco increased obviously in the 6th month point in PFD group (p=0.005, 0.004 and p <0.001 respectively). A lower ground glass score and fibrotic score in the PFD group approached statistical significance after 6-months treatment with pirfenidone when compared with control group (p=0.037 and 0.018, respectively) and decreased greatly in the 6th month point when compared to the former time points in PFD group (p=0.006 and 0.013, respectively), whereas no such changes were observed in the control group. Conclusion: These results suggest that pirfenidone could improve the pulmonary function tests and HRCT scores of connective tissue diseases related interstitial lung disease. It is a potential therapeutic candidate for treatment.