Successful treatment of a small cohort of patients with adult onset of Still's disease with infliximab: first experiences

Objective-To test the efficacy of infliximab in the treatment of patients w ith severe and active adult onset Still's syndrome (AOSD) despite conventio nal immunosuppressive therapy. Patients and methods-Six patients with the d iagnosis of AOSD according to the Yamagushi criteria of 1992 were treated w ith infliximab. All patients had severe disease with high clinical and sero logical activity. Patients were treated initially with high dose steroids or more intensive i mmunosuppressive therapy. Two patients had a history of multiple disease mo difying antirheumatic drug (DMARD) treatments. One patient had a history of three years of AOSD with fever, chills, pleural and pericardial effusions, and hepatosplenomegaly. Despite these treatments, he developed increasing serological signs of inflammation and arthritis of both hips and peripheral joints. Another patient had a history of five years of AOSD with oligoarth ritis, myalgias, and recurrent fever despite multiple DMARD treatment, incl uding cyclophosphamide pulse therapy. Our patients with AOSD presented with massive polyarthralgias, polyarthritis, splenomegaly or hepatomegaly, the typical rash, sore throat, weight loss, serositis, continuing fever, leucoc ytosis, and raised C reactive protein (CRP), erythrocyte sedimentation rate (ESR), and ferritin levels. Four patients with early onset of the disease, fulfilling the diagnostic criteria for AOSD and a clinical and serological high disease activity, were included in our pilot study without any furthe r DMARD treatment apart from the initial steroid treatment. Reduction of es tablished treatment, mainly with steroids, caused a relapse of the disease in all our patients. Patients then received 3-5 mg/kg infliximab on weeks 0 , 2, and 6, continuing with intervals of 6-8 weeks depending on the patient 's individual disease activity. Results-In all patients, fever, arthralgias, myalgias, hepatosplenomegaly, and the rash resolved after the first courses of treatment with infliximab. All serological variables (CRP, ESR, hyperferritinaemia) returned to norma l. After three courses of infliximab infusions, splenomegaly could not be d etected in any of our patients. One patient still had severe pain in the le ft hip caused by hip postarthritic osteoarthrosis, requiring hip replacemen t. After three courses of treatment with infliximab, splenomegaly could not be detected in any of our patients. Up to now, our patients have received infliximab infusion treatment for between five and 28 months. Throughout th is period all patients have continued to benefit from this treatment, with improvement in their clinical symptoms, joint counts, and serological disea se activity. One of our patients had a moderate infusion reaction during th e second treatment. The infusion was discontinued for one hour and then was resumed with no further problems. Conclusion-The disease improved remarkably in all six patients with AOSD af ter treatment with infliximab, also in the early stage of AOSD. These preli minary data suggest the potential therapeutic benefit of anti-tumour necros is factor a treatment in AOSD.