The decrease of surfactant protein D in bronchoalveolar lavage fluid in patients with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia

printing supported by . Visit Chiesi at Stand B2.10 TUESDAY, SEPTEMBER 4TH 2012 P3652 The efficacy of pirfenidone in scleroderma related interstitial lung disease Yukiko Miura1, Yoshiya Tsunoda1, Toru Tanaka1, Hiroyuki Takoi1, Yohei Yatagai1, Shigen Rin1, Akimasa Sekine1, Kenji Hayashihara1, Takefumi Saito1, Akihiko Gemma2, Arata Azuma2. 1Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Ibaraki, Japan; 2Division of Pulmonary Medicine, Infection and Oncology, Nippon Medical School, Internal Medicine, Tokyo, Japan 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. Case 2 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. P3653 Effect of pirfenidone on chronic interstitial pneumonia Hiroyuki Takoi1, Yukiko Miura2, Yoshiya Tsunoda1, Yuki Sumazaki1, Toru Tanaka1, Shih-Yuan Lin1, Yohei Yatagai1, Akimasa Sekine1, Kenji Hayashihara1, Takefumi Saito1. 1Department of Respiratory Medicine, Ibaraki-Higashi National Hospital, Ibaraki, Japan; 2Division of Pulmonary Medicine, Infectious Diseases, and Oncology, Dept. of Internal Medicine, Nippon Medical School, Tokyo, Japan Background: Several reports reveal the favorable effect of pirfenidone on early stage of idiopathic pulmonary fibrosis (IPF), but impacts on non-specific interstitial pneumonia (NSIP) and interstitial pneumonia (IP) associated with collagen vascular diseases (IP-CVD) are not clear. Objectives: To examine the effect of pirfenidone on chronic IP including IPF, NSIP and IP-CVD. Methods: Thirty-two patients were enrolled in the study evaluating the safety and efficacy of pirfenidone in IPF, NSIP and IP-CVD. Clinical diagnosis are IPF(14), NSIP(14), IP associated with scleroderma(3), and rheumatoid arthritis(1). Based on PaO2 at rest and SpO2 after 6 minutes walk test (6MWT), disease severity of those were classified into four groups [Table 1]. We retrospectively analyzed subjective symptom in British Medical Research Council scale, pulmonary function, KL-6, SP-D and CT findings before and during pirfenidone administration. Table 1. Disease severity of patients PaO2 at rest (mmHg) SpO2 after 6MWT n