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Abstract Number: 749

Treatment with Cyclophosphamide for Systemic Sclerosis-Interstitial Lung Disease Does Not Lead to a Sustained Improvement in Lung Function in Two Independent Cohorts

Elizabeth R. Volkmann1, Donald P. Tashkin1, Myung Sim1, Ning Li2, Dinesh Khanna3, Michael Roth4, Philip J. Clements4, Anna-Maria Hoffmann-Vold5, Daniel E. Furst1, Grace Kim6, Jonathan Goldin1 and Robert Elashoff7, 1University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, 2Biomathematics, University of California, Los Angeles, Los Angeles, CA, 3University of Michigan, Ann Arbor, MI, 4Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, 5Oslo University Hospital, Oslo, Norway, 6Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, 7University of California, Los Angeles, Los Angeles, CA

Meeting: ACR/ARHP Annual Meeting 2017

Date of first publication: September 18, 2017

Keywords: , ,

Session Information

Date: Sunday, November 5, 2017

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's – Clinical Aspects and Therapeutics Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: Compared with placebo, treatment with cyclophosphamide (CYC) improved lung function in patients with systemic sclerosis-related interstitial lung disease (SSc-ILD) after 1 year in Scleroderma Lung Study (SLS) I.1 However, the effects of CYC waned after monitoring patients for an additional year off therapy.2 In SLS II (comparing CYC and mycophenolate [MMF]), treatment with 1-year of CYC appeared to have a more sustained effect on lung function over 2-years, although SLS II used a different analysis approach than SLS I.3 To further understand the effects of CYC on SSc-ILD outcomes, the present analysis directly compared outcomes between the CYC arms of SLS I and II.

Methods: Participants enrolled in the CYC arms of SLS I (N=79) and II (N=73) were included. SLS I and II randomized participants to oral CYC for 1 year and followed patients for an additional year off therapy (In SLS II, patients received placebo in the second year). Eligibility criteria for SLS I and II were nearly identical. Outcomes included the FVC%-predicted and DLCO%-predicted (measured every 3 months) and quantitative radiographic extent of ILD (QILD) (measured at 1 and 2 years for SLS I and SLS II, respectively). Joint models were created to evaluate the treatment effect on the course of the FVC/DLCO over 2-years while controlling for baseline disease severity.

Results: SLS II-CYC participants had similar baseline characteristics compared with SLS I-CYC participants in terms of gender (75% vs. 77% female), disease duration (mean [SD] years: 2.6 [1.8] vs. 3.1 [2.3]), and FVC%-predicted (mean [SD]: 66.9 [9.9] vs. 67.6 [11.4]), respectively. SLS II-CYC patients were slightly older (mean [SD] years: 52.2 [9.6] vs. 48.4 [12.2]; P=0.037) and had a higher DLCO%-predicted (mean [SD]: 54.5 [14.6] vs. 47.2 [13.9]; P=0.0002) than SLS I-CYC participants. After adjusting for baseline QILD and FVC%-predicted, there was no difference in the course of the FVC%-predicted between the CYC arms of SLS I and II (P=0.535), nor the DLCO%-predicted (P=0.172). In both groups, treatment with CYC led to a significant improvement in the FVC%-predicted from 3-12 months, but no significant improvement beyond this point (Figure 1). Treatment with CYC had no effect on the DLCO for either group.

Conclusion: Although there are limitations in comparing participants from two trials, the baseline characteristics of the SLS I and SLS II participants were relatively similar. Treatment with 1 year of oral CYC led to similar improvements in lung function in both SLS I and II, although the effects were not sustained following CYC cessation. There results suggest that increasing the duration of ILD therapy may improve outcomes for SSc-ILD patients.

References:

1. NEJM 2006;354:2655-66.

2. Am J Respir Crit Care Med 2007;176:1026-34.

3. Lancet Resp Med 2016;4:708-19.

                                    Figure 1. Course of the FVC%-predicted by CYC treatment

                                    arm based on a joint model analysis.

 

Disclosure: E. R. Volkmann, None; D. P. Tashkin, None; M. Sim, None; N. Li, None; D. Khanna, Actelion, Bayer, BoehringerIngelheim, Chemomab, Corbus, Covis, Cytori,Eicos, EMD Serono, Genentech/Roche, Gilead, GSK, Sanofi-Aventis,UCB Pharma, 5,NIH/NIAMS, NIH/NIAID,Bayer, BMS, Genentech/Roche, Pfizer, 2,Eicos, 4; M. Roth, None; P. J. Clements, None; A. M. Hoffmann-Vold, None; D. E. Furst, None; G. Kim, None; J. Goldin, None; R. Elashoff, None.

To cite this abstract in AMA style:

Volkmann ER, Tashkin DP, Sim M, Li N, Khanna D, Roth M, Clements PJ, Hoffmann-Vold AM, Furst DE, Kim G, Goldin J, Elashoff R. Treatment with Cyclophosphamide for Systemic Sclerosis-Interstitial Lung Disease Does Not Lead to a Sustained Improvement in Lung Function in Two Independent Cohorts [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/treatment-with-cyclophosphamide-for-systemic-sclerosis-interstitial-lung-disease-does-not-lead-to-a-sustained-improvement-in-lung-function-in-two-independent-cohorts/. Accessed .

ACR Meeting Abstracts - https://acrabstracts.org/abstract/treatment-with-cyclophosphamide-for-systemic-sclerosis-interstitial-lung-disease-does-not-lead-to-a-sustained-improvement-in-lung-function-in-two-independent-cohorts/