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Autologous Hematopoietic Stem Cell Transplantation for Systemic Sclerosis: a Systematic Review and Meta-Analysis
Shouval, R., Furie, N., Raanani, P., Nagler, A., Gafter-Gvili, A.
Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation. 2018
Abstract
Autologous hematopoietic stem cell transplantation (AHSCT) has been proposed as a therapeutic modality for severe Systemic Sclerosis (SSc). We set to systematically review and meta-analyze the efficacy and safety of AHSCT in SSc. Randomized controlled trials (RCTs) and retrospective studies comparing AHSCT to standard immunosuppressive therapy were included. Of 363 titles screened from multiple databases, 15 were extracted for further investigation, and 4 met inclusion criteria (3 RCTs and 1 retrospective analysis). The control arm was monthly cyclophosphamide in all the RCTs and the majority of patients in the retrospective analysis (69%). Compared to the control, AHSCT reduced all-cause mortality (risk ratio [RR] 0.5 [95% CI 0.33-0.75]) and improved skin thickness (modified Rodnan Skin Score mean difference [MD] 10.62 [95% CI -14.21-7.03]), forced vital capacity (MD 9.58 [95% CI 3.89-15.18]), total lung capacity (MD 6.36 [95% CI 1.23-11.49]), and quality of life (physical SF-36 [MD 6.99 (95% CI 2.79-11.18)]). Treatment-related mortality considerably varied between trials but was overall higher with AHSCT (RR 9.00 [95%CI 1.57-51.69]). The risk of bias for studies included in the analysis was low. Overall, AHSCT reduces the risk of all-cause mortality and has properties of a disease-modifying anti-rheumatic treatment in SSc. Further investigation is warranted for refining patient selection and timing of transplantation.