Evolution of Systemic Sclerosis–Associated Interstitial Lung Disease One Year After Hematopoietic Stem Cell Transplantation or Cyclophosphamide
Arthritis Care & Research, vol. 74, pp. 433–441
Abstract
OBJECTIVE: Hematopoietic stem cell transplantation (HSCT) and cyclophosphamide (CYC) are treatment options for progressive systemic sclerosis associated with interstitial lung disease (SSc-ILD). The aims of our retrospective observational study were to evaluate: 1) the evolution of SSc-ILD in SSc patients treated with HSCT (assessed by high-resolution computed tomography [HRCT]; a group of patients treated with CYC was included as frame of reference); 2) how results of pulmonary function tests (PFTs) are associated with HRCT findings; and 3) which factors predict ILD reduction. METHODS: We semiquantitatively scored total ILD extent, reticulations, and ground-glass opacities (GGO) scores at baseline and at the 1-year HRCTs of SSc patients treated with HSCT or CYC. Linear association between changes in HRCT scores and PFT results and predictors of ILD improvement were studied. RESULTS: We included 51 patients (those treated with HSCT [n = 20] and those treated with CYC [n = 31]). The mean change in total ILD score was -5.1% (95% confidence interval [95% CI] -10.2, 0.0) in the HSCT treatment group (P = 0.050), and -1.0% (95% CI -4.3, 2.3) in the CYC treatment group (P = 0.535). For all patients, the evolution of HRCT scores was weakly associated with relative changes in PFT results. In univariate logistic regression, higher ground-glass opacities, higher total ILD, and lower single-breath diffusing capacity for carbon monoxide scores at baseline predicted improvement of ILD extent after treatment, but a multivariable model could not be built to assess independency of predictors. CONCLUSION: One year after treatment with HSCT, a nonsignificant but clear reduction of SSc-ILD extent was observed. Changes in PFT results were associated with changes in HRCT scores but the correlation was weak and cannot be considered conclusive.
Authors 8
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University of Modena and Reggio Emilia · Leiden University Medical Center · Azienda Ospedaliero-Universitaria di Modena · Istituto Ortopedico Rizzoli
Affiliation as printed
Leiden University Medical Center, Leiden, The Netherlands, Azienda Policlinico of Modena, University of Modena, and Reggio Emilia, Modena, Italy, and IRCCS Istituto Ortopedico Rizzoli Bologna Italy
Leiden University Medical Center, Leiden, The Netherlands, Azienda Policlinico of Modena, University of Modena, and Reggio Emilia, Modena, Italy, and IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
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Nina Marijn van Leeuwen Aachen
Leiden University Medical Center
Affiliation as printed
Leiden University Medical Center Leiden The Netherlands
Leiden University Medical Center, Leiden, The Netherlands
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Maaike Boonstra Aachen
Leiden University Medical Center
Affiliation as printed
Leiden University Medical Center Leiden The Netherlands
Leiden University Medical Center, Leiden, The Netherlands
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Lucia J.M. Kroft Aachen
Leiden University Medical Center
Affiliation as printed
Leiden University Medical Center Leiden The Netherlands
Leiden University Medical Center, Leiden, The Netherlands
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Anne A. Schouffoer Aachen
Leiden University Medical Center · Haga Hospital
Affiliation as printed
Leiden University Medical Center, Leiden, The Netherlands and Haga Teaching Hospital the Hague The Netherlands
Leiden University Medical Center, Leiden, The Netherlands and Haga Teaching Hospital, the Hague, The Netherlands
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Maarten K. Ninaber Aachen
Leiden University Medical Center
Affiliation as printed
Leiden University Medical Center Leiden The Netherlands
Leiden University Medical Center, Leiden, The Netherlands
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Tom W. J. Huizinga Aachen
Leiden University Medical Center
Affiliation as printed
Leiden University Medical Center Leiden The Netherlands
Leiden University Medical Center, Leiden, The Netherlands
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Leiden University Medical Center
Affiliation as printed
Leiden University Medical Center Leiden The Netherlands
Leiden University Medical Center, Leiden, The Netherlands
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