학술논문
Part 5: Allogeneic HSCT in refractory SJIA with lung disease; recent cases from centers in North America & Europe
Document Type
article
Author
Alexei A. Grom; Scott W. Canna; Rolla F. Abu-Arja; Rashmi Sinha; Luciana Peixoto; Elvira Cannizzaro; Shanmuganathan Chandrakasan; Kyla Driest; Rebecca Marsh; Bénédicte Neven; Karen Onel; Sampath Prahalad; Susan Prockop; Pierre Quartier; Johannes Roth; Grant Schulert; Juliana M.F. Silva; Donna Wall; Ulrike Zeilhofer
Source
Pediatric Rheumatology Online Journal, Vol 21, Iss S1, Pp 1-13 (2024)
Subject
Language
English
ISSN
1546-0096
Abstract
Abstract It 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.