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Bone Marrow Transplant. 2017 Nov;52(11):1495-1503. doi: 10.1038/bmt.2017.56. Epub 2017 May 22.

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.

Author information

1
Department of Internal Medicine, Unité Clinique de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, UF 04, Hôpital Saint-Louis, AP-HP Assistance Publique des Hôpitaux de Paris, INSERM UMRS 1160, Paris Denis Diderot University, Paris, France.
2
Department of Medicine, Division of Immunotherapy, Northwestern University, Chicago, IL, USA.
3
Departamento de Clínica Médica, Center for Cell-based Therapy, Regional Blood Center of Ribeirão Preto, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
4
Hôpital Européen Georges Pompidou, AP-HP Assistance Publique des Hôpitaux de Paris, INSERM UMR 970, Université Paris Descartes, Paris, France.
5
Department of Hematology, HSCT Unit, Hospital Clinic, Barcelona, Spain.
6
Department of Hematology, Saint-Antoine Hospital Paris, Assistance Publique des Hôpitaux de Paris, APHP, Paris, France.
7
Leiden University Medical Center, Leiden, The Netherlands.
8
Department of Rheumatology, Scleroderma Clinic, Osp. G. Pini, Milan, Italy.
9
Department of Hematology, Cord Blood Bank, Careggi University Hospital, Florence, Italy.
10
Division of Cardiology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
11
UCL Division of Medicine Royal Free Campus, London, UK.
12
Department of Rheumatology and Clinical Immunology, Charité University Medicine Berlin, Berlin, Germany.
13
Sheffield Pulmonary Vascular Disease Unit, M-floor, Royal Hallamshire Hospital, Sheffield, UK.
14
Department of Haematology, Sheffield Teaching Hospitals NHS Foundation Trust, University of Sheffield, Sheffield, UK.

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.

PMID:
28530671
PMCID:
PMC5671927
DOI:
10.1038/bmt.2017.56
[Indexed for MEDLINE]
Free PMC Article

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