Refractory adult-onset Still’s disease complicated with monoclonal gammopathy of undetermined significance: A case report

Abstract

Rationale: Adult-onset Still’s disease (AOSD) is a rare inflammatory disease characterized by a classic triad of daily spike fever, arthritis, and a typical salmon-pink rash. The involvement of inflammatory cytokines by various factors such as infection, drug, or neoplasm causes refractory AOSD. Patient concerns: We report a 63-year-old man with a high fever, rash, hyperferritinemia, and M proteinemia. His serum levels of interleukin-6 and interleukin-18 were remarkably high at 192 and 114,250 pg/mL, respectively. Diagnosis: AOSD complicated with monoclonal gammopathy of undetermined significance was diagnosed. Interventions: After steroid pulse therapy followed by oral prednisolone, cyclosporin, methotrexate, and colchicine, serum ferritin levels temporarily declined, but secondary cytomegalovirus infections exacerbated AOSD’s activity. Outcomes: Finally, after tocilizumab induction, AOSD activity was gradually suppressed over a long period. Lessons: The disease activity of AOSD is exacerbated by multiple factors, including comorbidities or infections. Clinicians need to consider that monoclonal gammopathy of undetermined significance complications might become AOSD refractory by an elevation of the inflammatory cytokines. Moreover, further prospective studies are required to confirm this result.