Abstract
Introduction: The major cause of death in Systemic Sclerosis (SSc) is interstitial lung disease, and cyclophosphamide is an only agent which significantly demonstrated a beneficial effect on lung function in patients with scleroderma-related interstitial lung disease (SSc-ILD), however the effect was quite modest, and it is necessary to identify a reasonable alternative.
Objectives: TGF-beta1 plays a critical role in the pathophysiology of pulmonary fibrogenesis. Pirfenidone exerts its antifibrotic effect through regulation of lung TGF-beta1 levels. This raises the possibility that agents targeting TGF-beta1 may be beneficial for SSc-ILD.
Methods: We administered pirfenidone to 3 patients with SSc-ILD and evaluated pulmonary function.
Results:
Case 1 is a 62 year-old female. Vital capacity (VC) improved by pirfenidone. The change rate was +27.3% (+0.51L) for 5 months.
Case2 is a 75 year-old female. VC improved remarkably, at the change rate of +44.4% (+0.32L) for 25 months.
Case 3 is a 66 year-old female. VC improved at the rate of +8.3% (+0.17L) for 26 months.
Conclusion: All of 3 patients with SSc-ILD demonstrated the favorable efficacy of VC by pirfenidone without severe adverse events. The previous studies documented that deteriorating lung function was associated with increased mortality in SSc-ILD. Therefore, it is necessary to identify and treat early stages of patients with SSc-ILD for the prevention of pulmonary function impairment. Pirfenidone exerts its antifibrotic effect through regulation of TGF-beta1, which is one of the important inducers of fibrogenesis in SSc. We suggest pirfenidone may be a possible option for SSc-ILD.
- © 2012 ERS