Adult-onset Still’s disease in the clinical practice of infectious disease physician

Abstract

Adult-onset Still’s disease (AOSD) is a rare multisystem disease of unknown etiology. According to the literature, AOSD incidence in the world is 0.1–0.4 per 100 thousand population and it has two age-related peaks: the first peak — young age (15–25 years), the second — in persons aged 35–46 years. The causes and pathogenetic mechanism of AOSD are not established. The infectious pathology nature has not received proper scientific confirmation. According to modern concepts, this disease belongs to the group of auto-inflammatory, occurring without autoimmune disorders. It was found that patients with AOSD have a T-cell regulation disorder, increased production of the proinflammatory cytokine, the long-term persistence of which leads to the occurrence of fever and aseptic inflammation in various organs and tissues. The main criteria for this nosology are young age, persistent fever, arthritis, maculopapular rash, elevated ferritin level, neutrophilic leukocytosis, seronegative rheumatoid arthritis. Concomitant diseases include polyserositis, lymphadenopathy, hepatosplenomegaly, and nasopharyngeal infection. The article presents a clinical case of AOSD verified in a young woman at the Infectious Diseases Clinical Hospital. The difficulties of diagnostics are described, taking into account the variety of symptoms and the absence of pathognomonic disease signs. KEYWORDS: Adult-onset Still’s disease, fever, auto-inflammatory disease, arthritis, maculopapular rash, ferritin. FOR CITATION: Kharlamova T.V., Smirnova T.Yu., Golub V.P. et al. Adult-onset Still’s disease in the clinical practice of infectious disease physician. Russian Medical Inquiry. 2020;4(11):705–709. DOI: 10.32364/2587-6821-2020-4-11-705-709.