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Michael E Weinblatt, Agnes L Maier, Steven S Overman, Philip J Mease,
Patricia A Fraser, Ellen M Gravallese Boston, MA and Seattle, WA

Rationale: Systemic onset juvenile rheumatoid arthritis (Still's Disease), an illness marked by recurrent high fever, skin rash, fatigue and
polyarthritis, may be progressive and refractory to conventional therapy.

 A randomized withdrawal trial of etanercept, the p75 TNF receptor fusion protein, demonstrated efficacy in polyarticular JRA (NEJM 342:763,2000) but it has not been studied in adult Still's disease. High levels of expression of both TNF and lymphotoxin a (LTa) have been noted in synovium of systemic onset JRA patients (A&R 39:1703,1996). Etanercept inhibits the activity of TNF and LTa making it an attractive therapeutic agent to use in Still's

Methods: This initial pilot study was designed as a six month open trial of etanercept in 12 adult patients who met the criteria for systemic onset juvenile rheumatoid arthritis and had active arthritis. This study was designed primarily to evaluate the effects of etanercept on the arthritis of Still's disease. Patients were initially dosed with etanercept 25 mg twice a
week, but the dose could be increased to 25 mg three times a week if no improvement in the arthritis was noted by week 8.

Results: The 12 patient's mean age was 36 yrs.(range 19-62 yrs)(10F:2M)with a disease duration of 10.7 yrs (1-17 yrs) and had received a mean of 3.6 (1-7)prior DMARDs. Five had received prior methotrexate and 5 remained on
methotrexate (15-25 mg/week) during the study. Ten were on prednisone at a
mean dose of 10 mg/day (5 -20 mg /day). At baseline they had a mean of 24▒11
painful joints, 19▒10 swollen joints and a mean ESR of 52▒38 mm/hr. Ten patients successfully completed the study and 2 withdrew due to disease flare at week 7 and at week 13. Four patients increased their etanercept from 25 biw to 25 tiw without any adverse events. Clinical response as
defined by ACR 20% response criteria was observed in 8 patients. Of these 8
responders, 5 met ACR 50% and two met ACR 70% responses. There was a 54%
improvement in painful joints, a 63% improvement in swollen joints and a 27%
improvement in sed rate. In the 3 patients who had systemic features of Still's (fever and rash) improvement in these features was seen in one patient. Except for the 2 pts who withdrew due to flare (rash, fever and arthritis) no other significant adverse events occurred.

Conclusions: In this initial study of Still's disease in the adult etanercept was well tolerated with improvement in the arthritis. This data suggests that additional trials should be performed to determine the effects
of TNF inhibitors on Still's disease.

Disclosure: M. Weinblatt, MD is a consultant to Immunex Corp.P.Mease, MD is
a consultant and on the speakers bureau of Immunex Corp. This research was
supported by a grant from Immunex Corp.

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