Scleroderma is an immune disease characterized by vascular abnormalities, inflammatory and progressive fibrosis of skin and internal organs. The worst prognosis, with high risk of early mortality, is in the case of visceral organ involvement (lung, digestive system and heart). The treatment is based on steroids, immunosuppressive medications but the prognosis is still very poor. Autologous stem cell transplantation (autoHSCT) still remains the promising option which can achieve long remission. In many years since the first autoHSCT in scleroderma almost 1000 patients have undergone the procedure. AutoHSCT is recommended with a grade I level of evidence for therapy of severe rapidly progressive scleroderma by the European Society for Blood and Marrow Transplantation ( EBMT ) Autoimmune Diseases Working Party ( ADWP ) guidelines.
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Anna Kopińska (2023) studied this question.
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