Part 5: Allogeneic HSCT in refractory SJIA with lung disease; recent cases from centers in North America & Europe

Author:

Grom Alexei A.,Canna Scott W.,Abu-Arja Rolla F.,Sinha Rashmi,Peixoto Luciana,Cannizzaro Elvira,Chandrakasan Shanmuganathan,Driest Kyla,Marsh Rebecca,Neven Bénédicte,Onel Karen,Prahalad Sampath,Prockop Susan,Quartier Pierre,Roth Johannes,Schulert Grant,Silva Juliana M.F.,Wall Donna,Zeilhofer Ulrike

Abstract

AbstractIt has been increasingly recognized that there is a subset of patients with refractory systemic JIA, who have failed all available medications and may benefit from HSCT. The increasing experience with HSCT in SJIA, suggests that despite the complicated post-HSCT course, short-term, the transplanted patients either achieved SJIA remission or reduced burden of disease. Longer follow-up, however, is needed to better define the long-term outcomes. The discussion at the NextGen 2022 conference was focused on the optimal timing for the procedure, the need for a good control of inflammatory SJIA activity prior to HSCT, and the role of the reduced intensity conditioning regimens as there was a remote concern that such regimens might increase the risk of SJIA relapse after the transplantation. There was unanimous agreement about the importance of long-term registries to address these questions.

Publisher

Springer Science and Business Media LLC

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