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

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.

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How I treat patients with adult onset Still's disease in clinical practice

Autoimmunity Reviews ◽

10.1016/j.autrev.2017.07.017 ◽

2017 ◽

Vol 16 (10) ◽

pp. 1016-1023 ◽

Cited By ~ 20

Author(s):

Marcello Govoni ◽

Alessandra Bortoluzzi ◽

Daniela Rossi ◽

Vittorio Modena

Keyword(s):

Clinical Practice ◽

Adult Onset Still’S Disease ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Adult Onset Still's Disease

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Diagnostic Challenge: A Report of Two Adult-Onset Still’s Disease Cases

Case Reports in Dermatological Medicine ◽

10.1155/2017/3768603 ◽

2017 ◽

Vol 2017 ◽

pp. 1-4 ◽

Cited By ~ 2

Author(s):

Sakunee Niranvichaiya ◽

Daranporn Triwongwaranat

Keyword(s):

Rheumatoid Factor ◽

Clinical Manifestations ◽

Maculopapular Rash ◽

Adult Onset Still’S Disease ◽

Adult Onset ◽

Still’S Disease ◽

Positive Rheumatoid Factor ◽

Salmon Pink ◽

Still's Disease ◽

Adult Onset Still's Disease

This study reports two adult-onset Still’s disease (AOSD) cases that met both Yamaguchi’s and Fautrel’s criteria and that presented with notable clinical manifestations. One case presented with atypical dermographism-like rash with an extremely high ferritin level. The other case presented with typical salmon-pink maculopapular rash but had atypical positive rheumatoid factor. This suggests that although negative rheumatoid factor is one of the criteria used for the diagnosis of AOSD, a positive rheumatoid factor result does not exclude AOSD. Beside a classic rash, characterized by transient salmon-pink maculopapular rash, we also find atypical dermographism-like rash. These findings remind us that there exist various types of rash from AOSD.

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Clinical Phenotypes of Adult-Onset Still’s Disease: New Insights from Pathophysiology and Literature Findings

Journal of Clinical Medicine ◽

10.3390/jcm10122633 ◽

2021 ◽

Vol 10 (12) ◽

pp. 2633

Author(s):

Stéphane Mitrovic ◽

Bruno Fautrel

Keyword(s):

Case Series ◽

Maculopapular Rash ◽

Epidemiological Studies ◽

Adult Onset Still’S Disease ◽

Polymorphonuclear Cells ◽

Adult Onset ◽

Still’S Disease ◽

Clinical Phenotypes ◽

Still's Disease ◽

Adult Onset Still's Disease

Adult-onset Still’s disease (AOSD) is a non-familial, polygenic systemic autoinflammatory disorder. It is traditionally characterized by four cardinal manifestations—spiking fever, an evanescent salmon-pink maculopapular rash, arthralgia or arthritis and a white-blood-cell count (WBC) ≥ 10,000/mm3, mainly neutrophilic polymorphonuclear cells (PMNs)—but many other manifestations and complications can be associated, making clinical expression very heterogeneous and diagnosis sometimes difficult. The AOSD course can be diverse and is currently impossible to predict. Several clinical phenotypes have been described, either on the basis of the evolution of symptoms over time (monocyclic, polycyclic and chronic evolution) or according to dominant clinical evolution (systemic and arthritis subtypes). However, these patterns are mainly based on case series and not on robust epidemiological studies. Furthermore, they have mainly been established a long time ago, before the era of the biological treatments. Thus, based on our personal experience and on recent advances in the understanding of disease pathogenesis, it appears interesting to reshuffle AOSD phenotypes, emphasizing the continuum between AOSD profiles and other systemic autoinflammatory disorders, eventually proposing a research agenda.

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