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HIGH DOSE CHEMOTHERAPY AND SYNGENEIC PROGENITOR CELL TRANSPLANTATION FOR SEVERE ADULT-ONSET STILL'S DISEASE

 

G.J. McColl1, J. Szer2, H. Kohsaka3, I.P. Wicks1

1Centre for Rheumatic Diseases; 2Bone Marrow Transplant Service, Royal Melbourne Hospital, Parkville Victoria 3050, Australia3Tokyo Medical and Dental University, Tokyo, Japan


The role of high dose chemotherapy and autologous peripheral blood progenitor cell (PBPC) transplant as treatment for severe autoimmune diseases, particularly rheumatoid arthritis, is currently the subject of considerable debate In this report we describe a man with severe adult-onset Still's disease who was treated with high dose chemotherapy and syngeneic PBPC transplantation.


A 38 year old identical twin presented with a seven year history of severe adult-onset Still's unresponsive to salazopyrine, methotrexate, methotrexate plus azathioprine and intramuscular gold and resulting in erosive destruction of wrist, ankle and shoulder joints and the persistent use of steroids. In March 1997, the affected twin consented, after prolonged discussion of the potential risks and benefits of the procedure, to undergo a syngeneic PBPC transplant. PBPCs were harvested from the unaffected twin after treatment with filgrastim. The affected twin was conditioned with cyclophosphamide and antithymocyte globulin (ATGAM) and haematopoietic rescue achieved with 2.59 × 106 CD34 + cells/kg from his unaffected brother. The transplant was complicated by a rash and a fever. The patient was discharged 14 days after the transplant and returned to regular exercise and fulltime work within 6 weeks. Eighteen months after the transplant the patient remains in complete remission with no swollen joints and a normal C-reactive protein. Prior to the PBPC transplant the twins circulating T lymphocyte Vb repertoires were distinctly different whereas after the transplant the affected twin, now in complete remission, has a Vb repertoire identical to that of the unaffected twin.


The use of high dose chemotherapy and autologous PBPC transplantation has recently been reported in three patients with severe rheumatoid arthritis, scleroderma and systemic lupus erythematosus. Our case is the first description of a prolonged remission in a patient with previously treatment-resistant adult-onset Still's disease after high dose chemotherapy and syngeneic, rather than autologous PBPC transplantation. The use of PBPCs from a genetically identical individual without autoimmune disease may increase the likelihood of "cure".

Disclosure: work reported in this abstract was supported by:
Arthritis Foundation of Victoria (Voluntary Self-Support Organization and Granting Agency.

Rheumatologic disorders: other
Prognostic factors and predictors of therapeutic response

Abstract: 1703
November 11, 1998
Poster Session F: RA Treatment
12:30-2:00 pm, Hall B1/C



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