Monocyclic form of adult-onset Still’s disease (case report)

K.Yu. Volkov, I.S. Svintsitskaya, A.O. Andriyanenko, M.M. Toporkov, D.S. Aganov, A.R. Belogurov, S.A. Klyuiev

S.M. Kirov Military Medical Academy, Saint-Petersburg

Adult-onset Still’s disease (AOSD) is an autoinflammatory disease classified by ICD-10 as other rheumatoid arthritis (M06.1). The incidence of AOSD in the world is 0.16 per 100 thousand annually. The etiology of this disease is unknown. There are following main AOSD forms: self-limiting (monocyclic), intermittent (polycyclic) and chronic articular. Patterns of clinical diagnosis are concerned with the absence of pathognomonic symptoms, as well as of rheumatoid factor and cyclic citrullinated peptide antibodies in the blood serum and synovial fluid. Thus, the diagnosis of AOSD is an exclusion diagnosis.

This paper describes a clinical case of AOSD progression in a male patient aged 20 years. The key problems and principles of differential diagnosis are considered in detail. The authors note that AOSD is a nosology difficult to diagnose, occurring under the mask of infectious intoxication syndrome with febrile fever and catarrhal manifestations in the upper respiratory tract. Differential diagnosis and determination of the fever genesis requires a large amount of laboratory and instrumental studies. The patient was diagnosed with AOSD after performing several studies that made it possible to exclude a number of diseases and on the basis of M. Yamaguchi (1992) 5 classification criteria, 2 of which were major (temperature rise more than 39 °C for at least 1 week; WBC more than 10.0×10⁶/L; neutrophilosis more than 80%), and 3 minor (sore throat, lymphadenopathy and splenomegaly, increased ALT and AST).

The authors demonstrated the efficacy of combination therapy with high doses of glucocorticosteroids (pulse methylprednisolone therapy with the switch to oral administration of 1 mg/kg/day) and methotrexate in the AOSD treatment. It was suggested that virus carriage (Epstein — Barr virus , cytomegalovirus, herpes viruses)in this patient might have triggered an auto-inflammatory process. The authors also noted that with correct and timely diagnosis, the monocyclic form is well amenable to therapy with the common achievement of persistent remission.

Keywords: fever of unknown origin, adult-onset Still’s disease, auto-inflammatory process, autoimmune pathology, neutrophilosis, hyperferritinemia, methylprednisolone, methotrexate.

For citation: Volkov K.Yu., Svintsitskaya I.S., Andriyanenko A.O. et al. Monocyclic form of adult-onset Still’s disease (case report). RMJ. 2020;7:28–32.