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Evolution of Systemic Sclerosis–Associated Interstitial Lung Disease One Year After Hematopoietic Stem Cell Transplantation or Cyclophosphamide
Author(s) -
Ciaffi Jacopo,
Leeuwen Nina M.,
Boonstra Maaike,
Kroft Lucia J. M.,
Schouffoer Anne A.,
Ninaber Maarten K.,
Huizinga Tom W. J.,
VriesBouwstra Jeska K.
Publication year - 2022
Publication title -
arthritis care and research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.032
H-Index - 163
eISSN - 2151-4658
pISSN - 2151-464X
DOI - 10.1002/acr.24451
Subject(s) - medicine , hematopoietic stem cell transplantation , interstitial lung disease , high resolution computed tomography , cyclophosphamide , dlco , gastroenterology , diffusing capacity , transplantation , pulmonary function testing , retrospective cohort study , univariate analysis , vital capacity , lung , surgery , multivariate analysis , chemotherapy , lung function
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.