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Adult-onset Still’s disease as a rare autoinflammatory disease (case report)

Abstract

Adult-onset Still’s disease as a rare autoinflammatory disease (case report)

T.K. Loginova1, A.A. Klimenko1,2, E.I. Shmidt1,2, I.A. Kovrigina1, A.A. Chinova1, A. Saikumar1 

1Pirogov Russian National Research Medical University, Moscow

2Pirogov City Clinical Hospital No. 1, Moscow 

Adult-onset Still’s disease (AOSD) is a rare autoinflammatory multisystem disease of unknown origin. Its principal symptoms are joint lesions, fever, skin rash, and neutrophilic leukocytosis in the absence of rheumatoid factor and cyclic citrullinated peptide antibodies in the blood and synovial fluid. AOSD is a systemic-onset type of juvenile rheumatoid arthritis that occurs in adults, usually between the ages of 15–25 and 36–45 years. The disease has no pathognomonic symptoms or specific laboratory and instrumental markers; therefore, it is a diagnosis of exclusion. This paper describes the case of a 21-year-old man with AOSD onset at the age of 17 years in the setting of physical activity and viral infection. Each exacerbation of AOSD in this patient included articular syndrome and severe systemic manifestations with high laboratory activity. A hallmark was an increase in the levels of ferritin and C-reactive protein at the time of exacerbation.The impossibility of total control disease with a basic drug required the prescription of a genetically engineered biological drug to ensure clinical and laboratory remission.

Keywords: adult-onset Still’s disease, autoinflammatory disease, pyrexia of unknown origin, juvenile rheumatoid arthritis, neutrophilic leukocytosis, ferritin.

For citation: Loginova T.K., Klimenko A.A., Shmidt E.I., Kovrigina I.A., Chinova A.A., Saikumar A. Adult-onset Still’s disease as a rare autoinflammatory disease (case report). RMJ. 2023;10:8–12.


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