فهرست نشریات علمی دسترسی آزاد ایران

Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disease characterized by spiking fever, arthralgia/arthritis and salmon-colored macular rashes. High-dose corticosteroids and methotrexate (MTX) are considered first-line treatments for AOSD. In refractory cases, targeted biologic therapies such as interleukin (IL)-1 and IL-6 inhibitors have demonstrated clinical benefit. The Janus kinase (JAK)–signal transducer and activator of transcription pathway mediate the signaling of multiple pro-inflammatory cytokines, and therefore represents a potential therapeutic target, particularly in refractory cases of AOSD. A 17-year-old woman presented with high-grade fever, arthralgia, profound fatigue, and transient skin lesions associated with episodes of fever. Despite treatment with high dose prednisolone, MTX, anakinra, adalimumab and etanercept, the disease was active. Therapy was initiated with baricitinib 4 mg/d in combination with leflunomide 20 mg/d and prednisolone dose was increased again to 30 mg/d. Fever subsided within several days, and arthritis was controlled over eight weeks. Leflunomide was discontinued due to oral ulceration. After nine months of treatment with baricitinib 4 mg/d, the disease remained in remission, and prednisolone was tapered to 2.5 mg/d. This report suggests that JAK inhibitors may be an effective treatment for treatment-resistant cases of chronic AOSD.