Adult-onset Still's Disease with Acute Kidney Injury Requiring Hemodialysis: A Case Report and Literature Review

Abstract

Adult-onset Still's disease (AOSD) is characterized by high spiking fever, evanescent rash, and arthritis. However, AOSD rarely presents with severe acute kidney injury (AKI). We herein present the case of a 56-year-old woman with new-onset AOSD who rapidly developed AKI. A physical examination and laboratory data revealed spiking fever, evanescent rash, thrombocytopenia, hyperferritinemia, and azotemia. The patient was diagnosed with AOSD complicated by AKI and macrophage activation syndrome. Treatment with high-dose steroids, hemodialysis, and plasma exchange successfully resolved her AKI. In this report, we review previously published reports on AOSD accompanied by AKI and discuss this rare complication in AOSD.

Keywords: Adult-onset Still's disease; acute kidney injury; hemodialysis.

Figure 1.

Clinical course of a 56-year-old woman with new-onset AOSD who rapidly developed AKI. Renal dysfunction gradually improved in parallel with a decrease in the serum ferritin level, and hemodialysis was discontinued approximately 2 weeks after admission. Moreover, proteinuria and hematuria gradually resolved. After continuing immunosuppressive therapy, the patient’s anemia and thrombocytopenia gradually improved. Renal replacement therapy was discontinued after a total of three hemodialysis cycles. The patient was discharged after 2 months of hospitalization. mPSL: methylprednisolone, BT: body temperature, PE: plasma exchange, HD: hemodialysis, Cre: creatinine

Figure 2.

Skin findings in a 56-year-old female patient with adult-onset Still’s disease. A) Pale erythema of the anterior chest, abdomen, and upper arms was observed before the start of treatment. B) The skin rash improved after the start of treatment and there were no flare-ups at 2 months after discharge.