Abstract
Autologous hematopoietic stem cell transplantation (aHSCT) represents an effective treatment for systemic sclerosis (SSc), but it also can cause immunological adverse events (iAEs). Therefore, we aimed to determine the frequency of iAEs [engraftment syndrome (ES) and secondary autoimmune disorder (sAD)] and to identify potential risk factors for their development in a retrospective analysis on 22 patients similarly transplanted due to SSc. While nine patients (41%) suffered from ESs, seven sADs occurred in six patients (27%). Patients who developed ES were older in our cohort (52.45 vs. 42.58 years, p = .0433, Cohen’s d = 0.86), and cardiac involvement by SSc was associated with development of ES (OR = 40.11, p = .0017). Patients with manifestation of sAD had a higher modified Rodnan skin score (mRSS) reduction after aHSCT (90.50% vs. 60.00%, p = .0064, r = .65). Thus, IAEs are common after aHSCT for SSc and can occur in different stages during and after aHSCT with characteristic clinical manifestations. Good cutaneous response after aHSCT might be considered as a risk factor for sAD, and higher age at aHSCT and cardiac involvement might be considered as risk factors for the development of ES.
Highlights
Since the ASSIST, ASTIS, and SCOT trials, autologous hematopoietic stem cell transplantation is an established therapeutic option in the treatment of life- or organ-threatening systemic sclerosis (SSc) [1,2,3,4,5,6,7,8]
We demonstrated that in SSc, Autologous hematopoietic stem cell transplantation (aHSCT) is associated with the development of different types of immunological adverse events (iAEs)
engraftment syndrome (ES) is present in aHSCT for other diseases, while the development of a secondary autoimmune disorder (sAD) seems to be a particular risk if transplantation is due to a primary autoimmune diseases (pADs)
Summary
Since the ASSIST, ASTIS, and SCOT trials, autologous hematopoietic stem cell transplantation (aHSCT) is an established therapeutic option in the treatment of life- or organ-threatening systemic sclerosis (SSc) [1,2,3,4,5,6,7,8]. During the engraftment period and iAEs After aHSCT in SSc the white blood cell (WBC) recovery, ES can endanger the patient [9,10,11,12,13]. These reactions are likely caused by the regeneration of leukocytes with following tissue invasion and triggering of autoinflammatory reactions [9,10,11,12,13].
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