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Summary  Children with systemic  J uvenile  I diopathic  A rthritis (s JIA ), the most severe subtype of  JIA , are at risk from destructive polyarthritis and growth failure, and corticosteroids as part of conventional treatment can result in osteoporosis and growth delay. In children where there is failure or toxicity from drug therapies, disease has been successfully controlled by  T ‐cell‐depleted autologous stem cell transplantation ( ASCT ). At present, the immunological basis underlying remission after  ASCT  is unknown. Immune reconstitution of  T  cells,  B  cells, natural killer cells, natural killer  T  cells and monocytes, in parallel with  T ‐cell receptor ( TCR ) diversity by analysis of the β variable region ( TCRV b) complementarity determining region‐3 ( CDR 3) using spectratyping and sequencing, were studied in five children with s JIA  before and after  ASCT . At time of follow up (mean 11·5 years), four patients remain in complete remission, while one child relapsed within 1 month of transplant. The  CD 8 +   TCRV b repertoire was highly oligoclonal early in immune reconstitution and re‐emergence of pre‐transplant  TCRV b  CDR 3 dominant peaks was observed after transplant in certain  TCRV b families. Further, re‐emergence of pre‐ ASCT  clonal sequences in addition to new sequences was identified after transplant. These results suggest that a chimeric  TCR  repertoire, comprising  T ‐cell clones developed before and after transplant, can be associated with clinical remission from severe arthritis.

immunology

Immunological characteristics and  T ‐cell receptor clonal diversity in children with systemic juvenile idiopathic arthritis undergoing  T ‐cell‐depleted autologous stem cell transplantation