Research ArticleArticle
Cyclophosphamide for Systemic Sclerosis-related Interstitial Lung Disease: A Comparison of Scleroderma Lung Study I and II
Elizabeth R. Volkmann, Donald P. Tashkin, Myung Sim, Ning Li, Dinesh Khanna, Michael D. Roth, Philip J. Clements, Anna-Maria Hoffmann-Vold, Daniel E. Furst, Grace Kim, Jonathan Goldin and Robert M. Elashoff
The Journal of Rheumatology February 2019, jrheum.180441; DOI: https://doi.org/10.3899/jrheum.180441
Elizabeth R. Volkmann
From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: evolkmann@mednet.ucla.edu. Accepted for publication October 18, 2018.
Donald P. Tashkin
From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: evolkmann@mednet.ucla.edu. Accepted for publication October 18, 2018.
Myung Sim
From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: evolkmann@mednet.ucla.edu. Accepted for publication October 18, 2018.
Ning Li
From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: evolkmann@mednet.ucla.edu. Accepted for publication October 18, 2018.
Dinesh Khanna
From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: evolkmann@mednet.ucla.edu. Accepted for publication October 18, 2018.
Michael D. Roth
From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: evolkmann@mednet.ucla.edu. Accepted for publication October 18, 2018.
Philip J. Clements
From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: evolkmann@mednet.ucla.edu. Accepted for publication October 18, 2018.
Anna-Maria Hoffmann-Vold
From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: evolkmann@mednet.ucla.edu. Accepted for publication October 18, 2018.
Daniel E. Furst
From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: evolkmann@mednet.ucla.edu. Accepted for publication October 18, 2018.
Grace Kim
From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: evolkmann@mednet.ucla.edu. Accepted for publication October 18, 2018.
Jonathan Goldin
From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: evolkmann@mednet.ucla.edu. Accepted for publication October 18, 2018.
Robert M. Elashoff
From the Department of Medicine and Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California; Department of Biomathematics, University of California, Los Angeles, California; Department of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA; Department of Rheumatology, Oslo University Hospital, Oslo, Norway. This work was supported by grants from the US National Heart, Lung, and Blood Institute/ National Institutes of Health (NIH): R01 HL089758 (DPT) and R01 HL089901 (RME), NIH/National Center for Advancing Translational Science, University of California, Los Angeles CTSI Grant Number UL1TR000124 (NL), the Scleroderma Foundation (ERV), and the Rheumatology Research Foundation (ERV). E.R. Volkmann, MD, MS, Assistant Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; D.P. Tashkin, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; M. Sim, PhD, Associate Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; N. Li, PhD, Associate Professor, Department of Biomathematics, University of California, Los Angeles; D. Khanna, MD, MS, Department of Medicine, Professor, University of Michigan Medical School; M.D. Roth, MD, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; P.J. Clements, MD, MPH, Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; A.M. Hoffmann-Vold, MD, PhD, Postdoctoral Candidate, Department of Rheumatology, Oslo University Hospital; D.E. Furst, MD, Emeritus Professor, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine; G. Kim, PhD, Associate Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; J. Goldin, MD, PhD, Professor, Department of Radiology, University of California, Los Angeles, David Geffen School of Medicine; R.M. Elashoff, PhD, Distinguished Professor, Department of Biomathematics, University of California, Los Angeles. Address correspondence to Dr. E.R. Volkmann, 1000 Veteran Ave., Ste. 32-59, Los Angeles, California 90095, USA. E-mail: evolkmann@mednet.ucla.edu. Accepted for publication October 18, 2018.
Abstract
Objective To compare safety and efficacy outcomes between the cyclophosphamide (CYC) arms of Scleroderma Lung Study (SLS) I and II.
Methods Participants enrolled in the CYC arms of SLS I (n = 79) and II (n = 69) 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 Year 2). Eligibility criteria for SLS I and II were nearly identical. Outcomes included the forced vital capacity (FVC%)-predicted and DLCO%-predicted (measured every 3 mos) and quantitative radiographic extent of interstitial lung disease (measured at 1 and 2 yrs 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 I and II CYC participants had similar baseline characteristics. After adjusting for baseline disease severity, there was no difference in the course of the FVC%-predicted (p = 0.535) nor the DLCO%-predicted (p = 0.172) between the SLS I and II CYC arms. In both groups, treatment with CYC led to a significant improvement in the FVC%-predicted from 3 to 12 months, but no significant improvement beyond this point. Treatment with CYC had no effect on the DLCO for either group.
Conclusion 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 cessation of CYC. These results suggest that increasing the duration of ILD therapy may improve outcomes for patients with systemic sclerosis–ILD.
In this issue
The Journal of Rheumatology
Vol. 51, Issue 12
1 Dec 2024
Cyclophosphamide for Systemic Sclerosis-related Interstitial Lung Disease: A Comparison of Scleroderma Lung Study I and II
Elizabeth R. Volkmann, Donald P. Tashkin, Myung Sim, Ning Li, Dinesh Khanna, Michael D. Roth, Philip J. Clements, Anna-Maria Hoffmann-Vold, Daniel E. Furst, Grace Kim, Jonathan Goldin, Robert M. Elashoff
The Journal of Rheumatology Feb 2019, jrheum.180441; DOI: 10.3899/jrheum.180441
Cyclophosphamide for Systemic Sclerosis-related Interstitial Lung Disease: A Comparison of Scleroderma Lung Study I and II
Elizabeth R. Volkmann, Donald P. Tashkin, Myung Sim, Ning Li, Dinesh Khanna, Michael D. Roth, Philip J. Clements, Anna-Maria Hoffmann-Vold, Daniel E. Furst, Grace Kim, Jonathan Goldin, Robert M. Elashoff
The Journal of Rheumatology Feb 2019, jrheum.180441; DOI: 10.3899/jrheum.180441
