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.

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.

Bone Marrow Transplant. 2017 May 22;:

Authors: Farge D, Burt RK, Oliveira MC, Mousseaux E, Rovira M, Marjanovic Z, de Vries-Bouwstra J, Del Papa N, Saccardi R, Shah SJ, Lee DC, Denton C, Alexander T, Kiely DG, Snowden JA, EBMT ADWP Scleroderma Working Group

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.Bone Marrow Transplantation advance online publication, 15 May 2017; doi:10.1038/bmt.2017.56.

PMID: 28530671 [PubMed - as supplied by publisher]

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