RT Journal Article
SR Electronic
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
FD The Journal of Rheumatology
SP 1064
OP 1072
VO 35
IS 6
A1 ALICE BÉREZNÉ
A1 BRIGITTE RANQUE
A1 DOMINIQUE VALEYRE
A1 MICHEL BRAUNER
A1 YANNICK ALLANORE
A1 DAVID LAUNAY
A1 VÉRONIQUE LE GUERN
A1 JEAN-EMMANUEL KAHN
A1 LOUIS-JEAN COUDERC
A1 JOËL CONSTANS
A1 PASCAL COHEN
A1 ALFRED MAHR
A1 CHRISTIAN PAGNOUX
A1 ERIC HACHULLA
A1 ANDRÉ KAHAN
A1 JEAN CABANE
A1 LOÏC GUILLEVIN
A1 LUC MOUTHON
YR 2008
UL http://www.jrheum.org/content/35/6/1064.abstract
AB 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.