Abstract: Objective To analyze the clinical features of Kawasaki Disease (KD) with arthritis and to explore the differential diagnosis between KD with arthritis and systemic juvenile idiopathic arthritis (SJIA). Methods The clinical data of two KD children with arthritis were retrospectively analyzed and the relevant literature was reviewed. Results One child was admitted witha high fever and abdominal pain complicated with gastrointestinal bleeding and macrophage activation syndrome (MAS), which was improved after hormone therapy. The child developed joint symptoms during the convalescence, and was considered as KD with arthritis. The other patient presented with fever and joint swelling and pain as the first symptoms. After treatment with two intravenous immunoglobulin (IVIG), intravenous methylprednisolone and methotrexate (MTX), the joint symptoms were not relieved significantly, but improved after treatment with recombinant human type II tumor necrosis factor receptor-antibody fusion protein (rhTNFR:Fc), and the child was considered as KD with JIA. Conclusions KD with arthritis and SJIA have high similarities in clinical manifestations. At present, there is no specific laboratory examination to distinguish the two diseases. Long-term clinical observation is needed to establish more precise diagnostic criteria and develop more experimental methods to assist diagnosis.

Key words: Kawasaki disease; arthritis; juvenile idiopathic arthritis; clinical feature