Adult-onset Stilĺs disease (AOSD) is a rare entity. The course of the disease can be mild or severe, acute or chronic. The intention of this survey was to evaluate the longterm efficacy of TNF blockade in patients with severe and active AOSD.
Eight patients with the diagnosis of AOSD, according to the diagnostic criteria developed by Yamaguchi in 1992, and pretreatment with either high dosage steroid or more intensive immunosuppressive therapy were treated with infliximab in a dosage of 3 - 5 mg/kg at time pointsduring week 0, 2 and 6. Later on, the treatment regimen was adapted to the individual needs of the patients. The follow-up period was between one and five years.
Seven patients responded to treatment with infliximab. Symptoms like fever, arthralgia, hepato-splenomegaly and serological parameters instantly improved. Five of these patients remained in remission over years after discontinuation of therapy. One of the responding patients needed permanent intensive treatment with TNF-blockers to control his severe chronic arthritis. Three patients experienced infusion reactions. One responding patient was therefore switched to etanercept and kept on this therapy. The one patient, who had not responded to infliximab also had no benefit from consecutive treatment with etanercept or adalimumab.
Anti-TNF therapy can have a lasting beneficial effect on the course of AOSD. Five of eight patients remained in remission even after termination of therapy.