Journalartikel
Autorenliste: Blank, Norbert; Schmalzing, Marc; Moinzadeh, Pia; Oberste, Max; Siegert, Elise; Mueller-Ladner, Ulf; Riemekasten, Gabriela; Guenther, Claudia; Koetter, Ina; Zeidler, Gabriele; Pfeiffer, Christiane; Juche, Aaron; Jandova, Ilona; Ehrchen, Jan; Susok, Laura; Schmeiser, Tim; Sunderkoetter, Cord; Distler, Jorg H. W.; Worm, Margitta; Kreuter, Alexander; Keysser, Gernot; Lorenz, Hanns-Martin; Krieg, Thomas; Hunzelmann, Nicolas; Henes, Jorg
Jahr der Veröffentlichung: 2022
Zeitschrift: Arthritis Research and Therapy
Bandnummer: 24
Heftnummer: 1
ISSN: 1478-6354
eISSN: 1478-6362
Open Access Status: Gold
DOI Link: https://doi.org/10.1186/s13075-022-02948-x
Verlag: BioMed Central
Abstract:
Background Current recommendations on the management of systemic sclerosis (SSc) suggest that autologous hematopoietic stem cell therapy (HSCT) can be a rescue therapy for patients with rapidly progressive SSc. Objectives To assess the safety and efficacy of HSCT for patients with SSc and to compare these with non-HSCT patients in a control cohort with adjusted risk factors. Methods A retrospective analysis of data from the multicentric German network for systemic scleroderma (DNSS) with 5000 patients with SSc. Control groups consisted of all patients with diffuse cutaneous (dc)-SSc (group A) and an adjusted high-risk cohort of male patients with Scl70-positive dc-SSc (group B). Results Eighty SSc patients received an HSCT 4.1 +/- 4.8 years after SSc diagnosis. Among them, 86.3% had dc-SSc, 43.5% were males, and 71.3% were positive for Scl70 antibodies. The control group A (n=1513) showed a significant underrepresentation of these risk factors for mortality. When the survival of the control group B (n=240) was compared with the HSCT group, a lower mortality of the latter was observed instead. Within 5 years after HSCT, we observed an improvement of the mRSS from 17.6 +/- 11.5 to 11.0 +/- 8.5 (p=0.001) and a stabilization of the DLCO. We did not see differences in transplant-related mortality between patients who received HSCT within 3 years after SSc diagnosis or later. Conclusion Our analysis of real-life data show that the distribution of risk factors for mortality is critical when HSCT cohorts are compared with non-HSCT control groups.
Zitierstile
Harvard-Zitierstil: Blank, N., Schmalzing, M., Moinzadeh, P., Oberste, M., Siegert, E., Mueller-Ladner, U., et al. (2022) Autologous hematopoietic stem cell transplantation improves long-term survival-data from a national registry, Arthritis Research and Therapy, 24(1), Article 258. https://doi.org/10.1186/s13075-022-02948-x
APA-Zitierstil: Blank, N., Schmalzing, M., Moinzadeh, P., Oberste, M., Siegert, E., Mueller-Ladner, U., Riemekasten, G., Guenther, C., Koetter, I., Zeidler, G., Pfeiffer, C., Juche, A., Jandova, I., Ehrchen, J., Susok, L., Schmeiser, T., Sunderkoetter, C., Distler, J., Worm, M., ...Henes, J. (2022). Autologous hematopoietic stem cell transplantation improves long-term survival-data from a national registry. Arthritis Research and Therapy. 24(1), Article 258. https://doi.org/10.1186/s13075-022-02948-x
Schlagwörter
Autologous hematopoietic stem cell transplantation; DIFFUSE SYSTEMIC-SCLEROSIS; DOUBLE-BLIND; GERMAN NETWORK FOR SYSTEMIC SCLERODERMA; PULSE CYCLOPHOSPHAMIDE; SCLERODERMA; SUBSETS; Systemic sclerosis
