Author:
Delplanque Marion,Mekinian Arsène,Georgin-Lavialle Sophie
Abstract
AbstractSugiyama 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.
Publisher
Springer Science and Business Media LLC
Reference6 articles.
1. Beck DB, Ferrada MA, Sikora KA, Ombrello AK, Collins JC, Pei W, et al. Somatic mutations in UBA1 and severe adult-onset autoinflammatory disease. N Engl J Med. 2020;383(27):2628–38.
2. Georgin-Lavialle S, Terrier B, Guedon AF, Heiblig M, Comont T, Lazaro E, et al. Further characterization of clinical and laboratory features occurring in VEXAS syndrome in a large-scale analysis of multicenter case-series of 116 French patients. Br J Dermatol. 2022;186(3):564–74.
3. Delplanque M, Aouba A, Hirsch P, Fenaux P, Graveleau J, Malard F, et al. USAID associated with myeloid neoplasm and VEXAS syndrome: two differential diagnoses of suspected adult onset Still’s disease in elderly patients. J Clin Med. 2021;10(23):5586.
4. van der Made CI, Potjewijd J, Hoogstins A, Willems HPJ, Kwakernaak AJ, de Sevaux RGL, et al. Adult-onset autoinflammation caused by somatic mutations in UBA1: a Dutch case series of patients with VEXAS. J Allergy Clin Immunol. 2021;S0091-6749(21):00819–8.
5. Mitrovic S, Fautrel B. Complications of adult-onset Still’s disease and their management. Expert Rev Clin Immunol. 2018;14(5):351–65.