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Reumatologia. 2019;57(1):50-54. doi: 10.5114/reum.2019.83240. Epub 2019 Feb 28.

Autologous hematopoietic stem cell transplant for progressive diffuse systemic sclerosis: procedural success and clinical outcome in 5-year follow-up.

Author information

1
Department of Rheumatology and Rehabilitation, Poznań University of Medical Sciences, Poland.
2
Department of Rheumatology and Osteoporosis, J. Struś Memorial Multidisciplinary Municipal Hospital in Poznań, Poland.
3
Student Department of Rheumatology, Metabolic Bone and Connective Tissue Disorders, Poznań University of Medical Sciences, Poland.
4
Department of Haematology and Stem Cell Transplantation, Poznań University of Medical Sciences, Poland.

Abstract

Systemic sclerosis is an autoimmune connective tissue disease affecting both skin and internal organs. Progressive disease with multiple organ involvement is considered to have a poor prognosis. Treatment possibilities are limited, but certain patients may benefit from autologous hematopoietic stem cell transplantation (auto-HSCT). We report a case of a 30-year-old woman with progressive diffuse systemic sclerosis treated with parenteral cyclophosphamide with unsatisfactory results. Due to progression of the disease and lack of alternative therapies auto-HSCT was performed. After instituting treatment with autologous hematopoietic stem cell transplantation no immunosuppressive therapy has been required during 5-year follow-up. Improvement in exertion tolerance, partial regression of skin lesions and stabilization of pulmonary and cardiovascular changes were observed. Currently therapeutic options in patients with progressive systemic sclerosis are limited. Hematopoietic stem cell transplantation might become an alternative therapeutic solution not only in the early phase of the disease but also among selected patients with progressive systemic sclerosis resistant to standard therapy.

KEYWORDS:

hematopoietic stem cells; progressive systemic sclerosis; scleroderma; stem cell transplantation

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