ArticleJournal of clinical medicine2021
USAID Associated with Myeloid Neoplasm and VEXAS Syndrome: Two Differential Diagnoses of Suspected Adult Onset Still's Disease in Elderly Patients.
Article in Journal of clinical medicine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 2 of them syntheses that pooled it.
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Who cites it
17 citing papers in PubMed, 2 syntheses or guidelines pooled it, 32 citations in OpenAlex.
- Pulmonary manifestations in VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome: a systematic review.Rheumatology international · 2023Pooled it
- Bibliometrics analysis on the research status and trends of adult-onset Still's disease: 1921-2021.Frontiers in immunology · 2022Pooled it
- Inflammasomes in Cardiovascular Diseases: Current Knowledge and Future Perspectives.International journal of molecular sciences · 2025Review
- Castleman's disease in the rheumatological practice.Zeitschrift fur Rheumatologie · 2024Review
- Role of allogeneic hematopoietic cell transplantation in VEXAS syndrome.Annals of hematology · 2024Review
- Still's Disease Onset in Older Adults: Clinical Features, Diagnosis, and Management.Drugs & aging · 2024Review
- [Castleman's disease in the rheumatological practice].Zeitschrift fur Rheumatologie · 2024Review
- Review
- Successful treatment with fludarabine and cyclophosphamide in a VEXAS syndrome patient with associated myelodysplastic syndrome: a case report and systematic review.Frontiers in oncology · 2024Article
- VEXAS Syndrome-Diagnostic Clues for the Dermatologist and Gaps in Our Current Understanding: A Narrative Review.JID innovations : skin science from molecules to population health · 2024Review
- Review
- Intrapatient competition of VEXAS syndrome and CML clones.Blood advances · 2023Article
- VEXAS syndrome: Current clinical, diagnostic and treatment approaches.Intractable & rare diseases research · 2023Review
- Safety and efficacy of canakinumab treatment for undifferentiated autoinflammatory diseases: the data of a retrospective cohort two-centered study.Frontiers in medicine · 2023Article
- Response letter to "Latent class analysis of 216 patients with adult-onset Still's disease" by Sugiyama et al.Arthritis research & therapy · 2022Article
- Article
- Commentary: 'Case Report: A Rare Case of Elderly-Onset Adult Onset Still's Disease in a Patient With Systemic Lupus Erythematous'.Frontiers in immunology · 2022Article
Corrections and comments
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Authors and funding
25 authors at 15 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPatients with solid cancers and hematopoietic malignancy can experience systemic symptoms compatible with adult-onset Still's disease (AOSD). The newly described VEXAS, associated with somatic UBA1 mutations, exhibits an overlap of clinical and/or biological pictures with auto inflammatory signs and myelodysplastic syndrome (MDS).
objectivesTo describe a cohort of patients with signs of undifferentiated systemic autoinflammatory disorder (USAID) concordant with AOSD and MDS/chronic myelomonocytic leukemia (CMML) and the prevalence of VEXAS proposed management and outcome.
methodsA French multicenter retrospective study from the MINHEMON study group also used for other published works with the support of multidisciplinary and complementary networks of physicians and a control group of 104 MDS/CMML.
resultsTwenty-six patients were included with a median age at first signs of USAID of 70.5 years with male predominance (4:1). Five patients met the criteria for confirmed AOSD. The most frequent subtypes were MDS with a blast excess (31%) and MDS with multilineage dysplasia (18%). Seven patients presented with acute myeloid leukemia and twelve died during a median follow-up of 2.5 years. Six out of 18 tested patients displayed a somatic UBA1 mutation concordant with VEXAS, including one woman. High-dose corticosteroids led to a response in 13/16 cases and targeted biological therapy alone or in association in 10/12 patients (anakinra, tocilizumab, and infliximab). Azacytidine resulted in complete or partial response in systemic symptoms for 10/12 (83%) patients including 3 VEXAS.
conclusionsSystemic form of VEXAS syndrome can mimic AOSD. The suspicion of USAID or AOSD in older males with atypia should prompt an evaluation of underlying MDS and assessment of somatic UBA1 mutation.
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