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.
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http://dx.doi.org/10.1186/s12969-023-00868-x | DOI Listing |
Introduction: Anakinra has dramatically improved the management of systemic juvenile idiopathic arthritis (SJIA) over the last decade. Nevertheless, management remains inconsistent; corticosteroids are still frequently used. We analyzed the course of SJIA in children treated with anakinra according to the time of treatment initiation after disease onset.
View Article and Find Full Text PDFPediatr Rheumatol Online J
December 2024
Infection, Immunity and Global Health Theme, Murdoch Children's Research Institute, Parkville, VIC, 3052, Australia.
Background: Juvenile idiopathic arthritis (JIA) is challenging to classify and effectively monitor due to the lack of disease- and subtype-specific biomarkers. A robust molecular signature that tracks with specific JIA features over time is urgently required, and targeted plasma metabolomics may reveal such a signature. The primary aim of this study was to characterise the differences in the plasma metabolome between JIA patients and non-JIA controls and identify specific markers of JIA subtype.
View Article and Find Full Text PDFLancet Rheumatol
December 2024
Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA; Pharming Healthcare, Warren, NJ, USA. Electronic address:
Background: Systemic juvenile idiopathic arthritis-related lung disease (sJIA-LD) is a severe complication in patients with treatment-refractory systemic juvenile idiopathic arthritis (sJIA). The objective of this study was to evaluate the effect of allogeneic haematopoietic stem-cell transplantation (HSCT) in a cohort of children with sJIA-LD.
Methods: This international, retrospective cohort study was performed in nine hospitals across the USA and Europe in children with sJIA-LD who had received allogeneic HSCT.
SAGE Open Med Case Rep
December 2024
Department of Pediatric Rheumatology, Imam Hossein Children's Hospital, Isfahan University of Medical Sciences, Isfahan, Iran.
Arthritis in children has several causes, including infectious, inflammatory, and infiltration of malignant cells, and it is important to differentiate them as soon as possible. We present the case of a 3-year-old boy who was initially diagnosed with septic arthritis secondary to joint pain, swelling, and high fever. Despite appropriate antibiotic therapy, the patient's symptoms persisted, leading to an eventual diagnosis of systemic juvenile idiopathic arthritis (sJIA).
View Article and Find Full Text PDFFront Pediatr
November 2024
Pediatric Rheumatology Department, Hospital Infantil de México Federico Gómez, Mexico City, Mexico.
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