Arthritis care & research · 2026
Current State of Allogeneic Hematopoietic Cell Transplantation for Still's Disease: A Collaborative Perspective from Attendees of the Systemic Juvenile Idiopathic Arthritis Foundation NextGen Conference.
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Chandra Sharat, Marsh Rebecca A, Silva Juliana M F, Wobma Holly, Prockop Susan, Slatter Mary, Henderson Lauren A, Schulert Grant S, Grom Alexei, Sinha Rashmi, Connelly James A
Аннотация
OBJECTIVE: Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) represent a spectrum of Still's disease (SD) characterized by dysregulated innate immunity, severe systemic inflammation, and risk of life-threatening complications including macrophage activation syndrome (MAS) and associated lung disease (LD). Although IL-1 and IL-6 pathway inhibitors have improved outcomes, a subset of patients develop severe, refractory disease. This article summarizes the current evidence and multidisciplinary expert perspectives on the role of allogeneic hematopoietic cell transplantation (HCT) in refractory Still's disease.
METHODS: This Special Article is a conference summary based on presentations and multidisciplinary discussion at the 2024 Systemic JIA Foundation NextGen Conference, which convened an international, multidisciplinary group of rheumatologists, transplant physicians, immunologists, pulmonologists, and patient advocates.
RESULTS: Recurrent MAS, progressive LD, persistent systemic inflammation despite IL-1/IL-6/JAK inhibition, and glucocorticoid dependence were identified as clinical features that may warrant consideration of allogeneic HCT in SD. Available data, mainly from small pediatric cohorts, suggest that allogeneic HCT can achieve clinical remission and improve pulmonary outcomes in select patients. However, transplant-related mortality remains substantial, particularly in patients with LD, and inflammatory disease may recur after HCT. HCT data in adults remain limited.
CONCLUSION: Allogeneic HCT may be considered for select patients with severe, refractory SD, particularly those with recurrent MAS and/or progressive LD. Early multidisciplinary evaluation, shared decision-making, and careful patient selection are essential. Prospective multicenter studies and closer collaboration between pediatric and adult providers are needed to refine patient selection and improve outcomes.
METHODS: This Special Article is a conference summary based on presentations and multidisciplinary discussion at the 2024 Systemic JIA Foundation NextGen Conference, which convened an international, multidisciplinary group of rheumatologists, transplant physicians, immunologists, pulmonologists, and patient advocates.
RESULTS: Recurrent MAS, progressive LD, persistent systemic inflammation despite IL-1/IL-6/JAK inhibition, and glucocorticoid dependence were identified as clinical features that may warrant consideration of allogeneic HCT in SD. Available data, mainly from small pediatric cohorts, suggest that allogeneic HCT can achieve clinical remission and improve pulmonary outcomes in select patients. However, transplant-related mortality remains substantial, particularly in patients with LD, and inflammatory disease may recur after HCT. HCT data in adults remain limited.
CONCLUSION: Allogeneic HCT may be considered for select patients with severe, refractory SD, particularly those with recurrent MAS and/or progressive LD. Early multidisciplinary evaluation, shared decision-making, and careful patient selection are essential. Prospective multicenter studies and closer collaboration between pediatric and adult providers are needed to refine patient selection and improve outcomes.