BARBARA ANSELL SERIES

Rheumatology

Autologous haematopoietic stem cell transplantation in juvenile idiopathic arthritis

L R Wedderburn¹, M Abinun², P Palmer³, H E Foster⁴

¹ Rheumatology Unit, Institute of Child Health, UCL and Great Ormond Street Hospital NHS Trust, London, UK
² Department of Paediatric Immunology, Newcastle General Hospital, Newcastle Hospitals NHS Trust, Newcastle upon Tyne, UK
³ Children’s Bone Marrow Transplantation Unit, Newcastle General Hospital, Newcastle Hospitals NHS Trust, Newcastle upon Tyne, UK
⁴ Departments of Rheumatology and Child Health, University of Newcastle upon Tyne and Newcastle Hospitals NHS Trust, Newcastle upon Tyne, UK

Correspondence to:
Correspondence to:
Dr L R Wedderburn, Rheumatology Unit, Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK;
l.wedderburn@ich.ucl.ac.uk

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A powerful strategy when other treatments have failed

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Keywords: juvenile idiopathic transplantation; autologous haematopoietic stem cell transplantation; autoimmune disease

Abbreviations: ASCT, autologous stem cell transplantation; ATG, antithymocyte globulin; BMT, bone marrow transplantation; CMV, cytomegalovirus; DMARDs, disease modifying antirheumatic drugs; EBV, Epstein Barr virus; GVHD, graft versus host disease; G-CSF, granulocyte-colony stimulating factor; JIA, juvenile idiopathic arthritis; MAS, macrophage activation syndrome; MS, multiple sclerosis; NSCAG, National Specialist Commisionary Advisory Group; PBSC, peripheral blood stem cells; RA, rheumatoid arthritis; SLE, systemic lupus erythematosus; SOJIA, systemic onset JIA; TBI, total body irradiation

The first 150 words of the full text of this article appear below.

Despite modern therapies, juvenile idiopathic arthritis (JIA) remains a significant cause of morbidity in children. The long term outcome of JIA is variable. Thus, while the prognosis for many children with JIA is good, over one third of children have ongoing active disease into adulthood with sequelae from chronic inflammation.^1,2 Patients with systemic or polyarticular onset JIA tend to have a worse prognosis, even with the early use of disease modifying antirheumatic drugs (DMARDS). These patients have considerable morbidity from joint damage, osteoporosis, growth retardation, psychosocial morbidity, reduced quality of life, and educational or employment disadvantage.^2,3

The concept of treating severe autoimmune disease by using intense, high dose immunosuppression to remove autoreactive lymphocytes, followed by rescue with haematopoietic stem cells, is based on the tenet that the immune system plays a pivotal role in such diseases. Support for this concept came from animal models, in which both spontaneous and . . . [Full text of this article]

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This article has been cited by other articles:

• Foster, H., Davidson, J., Baildam, E., Abinun, M., Wedderburn, L. R., on behalf of the British Society for Paediatric an, (2006). Autologous haematopoeitic stem cell rescue (AHSCR) for severe rheumatic disease in children: Guidance for BSPAR members--executive summary. Rheumatology (Oxford) 45: 1570-1571 [Full Text]  
• Goldmuntz, E. A., White, P. H. (2006). Juvenile Idiopathic Arthritis:: A Review for the Pediatrician. Pediatr. Rev. 27: e24-e32 [Full Text]  
• (2005). Rheumatology. Arch. Dis. Child. 90: A51-A53 [Full Text]  
• (2003). Rheumatologists are called to collaborate in treating childhood JIA. Ann Rheum Dis 62: 443-443 [Full Text]