Cardiopulmonary assessment of patients with systemic sclerosis for hematopoietic stem cell transplantation: Recommendations from the European Society for Blood and Marrow Transplantation Autoimmune Diseases Working Party and collaborating partners

D. Farg*, R. K. Burt, M. C. Oliveira, E. Mousseau, M. Rovir, Z. Marjanovi, J. De Vries-Bouwstr, N. Del Pap, R. Saccard, S. J. Shah, D. C. Lee, C. Dento, T. Alexande, D. G. Kiely, J. A. Snowden

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

45 Scopus citations

Abstract

Systemic sclerosis (SSc) is a rare disabling autoimmune disease with a similar mortality to many cancers. Two randomized controlled trials of autologous hematopoietic stem cell transplantation (AHSCT) for SSc have shown significant improvement in organ function, quality of life and long-term survival compared to standard therapy. However, transplant-related mortality (TRM) ranged from 3-10% in patients undergoing HSCT. In SSc, the main cause of non-transplant and TRM is cardiac related. We therefore updated the previously published guidelines for cardiac evaluation, which should be performed in dedicated centers with expertize in HSCT for SSc. The current recommendations are based on pre-transplant cardiopulmonary evaluations combining pulmonary function tests, echocardiography, cardiac magnetic resonance imaging and invasive hemodynamic testing, initiated at Northwestern University (Chicago) and subsequently discussed and endorsed within the EBMT ADWP in 2016.

Original languageEnglish (US)
Pages (from-to)1495-1503
Number of pages9
JournalBone Marrow Transplantation
Volume52
Issue number11
DOIs
StatePublished - Nov 1 2017

ASJC Scopus subject areas

  • Hematology
  • Transplantation

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