Autologous Hematopoietic Stem Cell Transplantation in Systemic Sclerosis: Clinical Cases and Literature Review

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Wed, 2019/09/11 - 12:12
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Laboratorinė medicina. 2019,
t. 21,
Nr. 2,
p. 75 -
83

Systemic sclerosis is a clinically heterogeneous multisystem autoimmune disorder driven by autoimmune inflammation, fibrosis and a microangiopathic vasculopathy. Raynaud’s phenomenon is often the first sign of the disease. The other signs such as sclerodactyly, ulcers on fingers, calcinosis and telangiectasia usually appear a few months later. Inflammation and fibrosis can also affect internal organs such as heart, lungs, kidney and digestive system. The statistics show that 47-56% of patients, who had been diagnosed with diffuse form of systemic sclerosis and the impact on internal organs (heart and lung), have lived for three years. The basic treatment of systemic sclerosis is not alwdoes not stop the development of the disease. Since 1997 the autologous hematopoietic stem cell transplantation has been evolved as a specific treatment for patients with systemic sclerosis after discovering that conventional treatment has not been effective for them. The randomized controlled tests have shown that transplantation is a more effective method of treatment compared with intravenous cyclophosphamide. After this treatment has been used, the statistics shows the reduce of the development of the disease, the improved ąuality of life and better long-term survival. Improved skin condition is reflected by a significant reduction of the modified Rodnan skin score and positive changes of lung functional tests correlate with improved patient clinical status, lower dyspnoea. The aim of this article is to analyze literature data on indications and contraindications of autologous hematopoietic stem cell transplantation, evaluate clinical studies performed in patients with systemic sclerosis, and to review the results of the Vilnius University Hospital Santaros Klinikos Center of Rheumatology.

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