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Background: In the past 20 years, hematopoietic stem cell transplantation (HSCT) has been investigated as treatment for systemic sclerosis (SSc). The goal of HSCT is to eradicate the autoreactive immune system, which is replaced by a new immune repertoire with long-lasting regulation and tolerance to autoantigens. Here, we describe the clinical outcomes of severe and refractory SSc patients that underwent HSCT at a single Brazilian center., Patients and Methods: This is a longitudinal and retrospective study, including 70 adult SSc patients, with an established diagnosis of SSc, and who underwent autologous HSCT from 2009 to 2016. The procedure included harvesting and cryopreservation of autologous hematopoietic progenitor cells, followed by administration of an immunoablative regimen and subsequent infusion of the previously collected cells. Patients were evaluated immediately before transplantation, at 6 months and then yearly until at least 5-years of post-transplantation follow-up. At each evaluation time point, patients underwent clinical examination, including modified Rodnan's skin score (mRSS) assessment, echocardiography, high-resolution computed tomography of the lungs and pulmonary function., Results: Median (range) age was 35.9 (19-59), with 57 (81.4%) female and median (range) non-Raynaud's disease duration of 2 (1-7) years. Before transplantation, 96% of the patients had diffuse skin involvement, 84.2%, interstitial lung disease and 67%, positive anti-topoisomerase I antibodies. Skin involvement significantly improved, with a decline in mRSS at all post-transplantation time points until at least 5-years of follow-up. When patients with pre-HSCT interstitial lung disease were analyzed, there was an improvement in pulmonary function (forced vital capacity and diffusing capacity of lung for carbon monoxide) over the 5-year follow-up. Overall survival was 81% and progression-free survival was 70.5% at 8-years after HSCT. Three patients died due to transplant-related toxicity, 9 patients died over follow-up due to disease reactivation and one patient died due to thrombotic thrombocytopenic purpura., Conclusions: Autologous hematopoietic progenitor cell transplantation improves skin and interstitial lung involvement. These results are in line with the international experience and support HSCT as a viable therapeutic alternative for patients with severe and progressive systemic sclerosis.

Aims: To identify clinical symptoms and nursing interventions for stem cell therapy in autoimmune diseases., Design: This is a retrospective, cross-sectional study., Methods: This study was undertaken with patients diagnosed with type 1 diabetes or multiple sclerosis, undergoing autologous haematopoietic stem cell transplantation from January 2004 - December 2018. Data were registered in a questionnaire, taken during the conditioning regimen comprising cyclophosphamide and rabbit anti-thymocyte globulin. Descriptive statistics and Fisher's exact test were used for data analysis., Results: There were 68 and 23 patients in the multiple sclerosis and type 1 diabetes groups respectively. Skin rash, nausea, vomiting and fever were more frequent and diverse in the type 1 diabetes group. Steroids were used as prophylaxis for anti-thymocyte globulin-associated allergic reactions in 97% of multiple sclerosis patients. Most of the identified symptoms and nursing interventions were more associated with one or other disease group (p < .05) and were more frequent in the type 1 diabetes group., Conclusion: Patients with autoimmune diseases who underwent stem cell therapy present differences in their repertoire of adverse events and require disease-specific nursing actions., Impact: Our results may enable nurses to establish transplant and disease-specific guidelines to improve prevention and management of adverse events and therefore optimize patient care and therapeutic success., (© 2020 John Wiley & Sons Ltd.)

Objective: describe the development of a virtual learning object to provide information about autologous transplantation of hematopoietic stem cells to autoimmune diseases., Methods: methodological study of a website development, using the instructional design model that includes Analysis, Design, Development and Implementation., Results: the virtual object, available at http://www.transplantardai.com.br, was developed in a web platform, in the Hypertext Markup Language, using the software WebAcappella - Responsive Website Creator (Intuisphere, France 2016). The content was structured in the modules: History, Transplant, Autoimmune Diseases, Links, Guidelines, Speech Team and Doubts. The icons and menus were created in order to attract the user, facilitating the search for information and allowing maximum use of the resources available on the website., Conclusion: the methodology used allowed the development of the virtual learning object, which can be used as a tool to guide and disseminate knowledge about this treatment.