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Sugiyama et al. recently described in "Latent class analysis of 216 patients with adult-onset Still's disease," baseline characteristics, laboratory tests, treatment, relapse, and death of adult-onset Still's disease (AOSD) patients from a Japanese hospital. They identified two subgroups: Class 1 (n=155) with a younger age and typical symptoms of AOSD and Class 2 (n=61) with older patients and fewer typical symptoms of AOSD. In 2022, VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, an established X-linked disease associated with a somatic mutation in UBA1, is considered as a differential diagnosis for AOSD particularly in elderly. These patients from Class 2 could benefit from more explorations for mild myelodysplasia and VEXAS.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9795768PMC
http://dx.doi.org/10.1186/s13075-022-02984-7DOI Listing

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