| Abstract [eng] |
Systemic sclerosis is a systemic autoimmune disease that manifests itself in varying degrees of damage to the skin and internal organs, sometimes leading to exhausting symptoms and life-threatening organ failure. In some cases, patients with severe and rapidly progressive disease have an inadequate response to first-line immunomodulatory therapy. In this case, autologous hematopoietic stem cell transplantation is proposed. This procedure is an innovative, clinically proven treatment for systemic sclerosis that reduces the risk of death, improves long-term lung and heart function, and improves the overall clinical condition. However, due to the infrequent use of such therapies, there is currently not enough data on adequate patient selection, transplant conditioning, cell preparation regimens, and complication prevention measures that should be used in the treatment of systemic sclerosis. In addition, this method of treatment poses several problems as it is associated with an increased risk of developing cardiological, pulmonological, infectious, autoimmune, oncological and other complications and requires specific preparation and long-term patient care during the post-transplantation period. In this masters thesis we describe a clinical case of a patient who underwent autologous hematopoietic stem cell transplantation due to pathological skin changes, worsening dyspnea, impaired lung function, and fibrous lesions seen on computed tomography despite initial treatment. This procedure went without severe complications, and after the treatment there was a marked improvement in the condition of the skin and lungs, which remained stable for a long time. Also, in this masters thesis we present a literature review of the outcomes of treating systemic sclerosis with autologous hematopoietic stem cell transplantation and discuss how they depend on the patient's condition prior to transplantation, the preparation for the procedure and immunosuppression protocols as well as patient care during long-term follow-up. |
