TY - JOUR T1 - Therapeutic Strategy Combining Intravenous Cyclophosphamide Followed by Oral Azathioprine to Treat Worsening Interstitial Lung Disease Associated with Systemic Sclerosis: A Retrospective Multicenter Open-label Study JF - The Journal of Rheumatology JO - J Rheumatol SP - 1064 LP - 1072 VL - 35 IS - 6 AU - ALICE BÉREZNÉ AU - BRIGITTE RANQUE AU - DOMINIQUE VALEYRE AU - MICHEL BRAUNER AU - YANNICK ALLANORE AU - DAVID LAUNAY AU - VÉRONIQUE LE GUERN AU - JEAN-EMMANUEL KAHN AU - LOUIS-JEAN COUDERC AU - JOËL CONSTANS AU - PASCAL COHEN AU - ALFRED MAHR AU - CHRISTIAN PAGNOUX AU - ERIC HACHULLA AU - ANDRÉ KAHAN AU - JEAN CABANE AU - LOÏC GUILLEVIN AU - LUC MOUTHON Y1 - 2008/06/01 UR - http://www.jrheum.org/content/35/6/1064.abstract N2 - Objective To evaluate the effects and safety of 6-month intravenous cyclophosphamide (CYC) followed by 18-month oral azathioprine (AZA) therapy in patients with systemic sclerosis (SSc) and worsening interstitial lung disease (ILD). Methods All patients presented with ILD and worsened forced vital capacity (FVC) and/or total lung capacity of more than 10% and/or DLCO of more than 15% during the previous year. Treatment was 6 monthly pulses of 0.6 g/m2 CYC followed by oral AZA for 18 months on disease stabilization or improvement. The endpoint was the rate of percentage change in pulmonary function tests (PFT) after 6 and 24 months. Results Twenty-seven patients with SSc (20 females) were recruited. Age and disease duration before CYC therapy were (mean ± SD) 49.4 ± 15 years and 75.5 ± 87.8 months, respectively. Mean baseline FVC was 67% ± 19% of predicted value. At 6 months, in 7 (26%) patients disease was improved, in 12 (44%) stabilized, and in 8 (30%) worsened. Among the 19 (70%) responders, 15 received AZA and 4 declined. Twenty-three completed 2-year followup, 3 died, and one dropped out. Six (22.2%) had improved, 8 (29.6.%) were stable, and 13 (48.2%) had worsened. Evolution of the slope of FVC (in % per year) varied from –15.5 prior to treatment to +3 (p = 0.004) at 6 months and to +1 (p < 5 ×10−5) at 24 months. Conclusion Intravenous CYC followed by oral maintenance immunosuppressive therapy for worsening ILD was well tolerated and was associated with stable or improved PFT in 70% and 51.8% of SSc patients at 6 months and 2 years, respectively. ER -