発熱と咽頭痛で発症した成人Still病例

抄録

Adult-onset Still’s disease (AOSD) is a multisystemic autoimmune disease characterized by fever, arthritis, and skin rash, with negative test results for rheumatoid factor and antinuclear antibody. Diagnosis of AOSD is often difficult because of the lack of specific symptoms and laboratory findings.

We report the case of a 39-year-old woman with AOSD who presented to our Department of Otorhinolaryngology with fever and sore throat. The patient was treated with antibacterial drugs and a steroid for acute pharyngitis. During the steroid taper, the fever and sore throat flared up again, and the patient developed signs of liver damage, a skin rash, and arthralgia. She was suspected as having AOSD because of marked elevation of the serum ferritin level and negative tests for antinuclear antibody and rheumatoid factor. After ruling out infection, malignancy, and other collagen diseases, we confirmed the diagnosis of AOSD. The patient developed macrophage activation syndrome, which improved following treatment with immunosuppressive drugs and IL-6 antagonists.

Fever and sore throat are often treated as upper respiratory infection in the field of otorhinolaryngology. However, in patients with other systemic symptoms such as skin rash and arthralgia who also show a poor response to antibacterial drugs, it is essential to consider the possibility of AOSD in the differential diagnosis. The prognosis of AOSD is generally good; however, as the disease could become severe in some cases, it is necessary to collaborate with a rheumatologist as early as possible during the management of these patients.