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[VEXAS-like auto inflammatory syndrome: 2 cases].
Rev Med Interne
December 2024
Service de médecine interne, CHI Poissy-St Germain, 10, rue du Champs Gaillard, 78300 Poissy, France.
Introduction: VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic), recently described, due to a somatic mutation of the UBA1 gene and often associated with hemopathy, is characterized by systemic symptoms close to those described in Still's disease or relapsing polychondritis. There are also patients with hemopathy, presenting inflammatory symptoms reminiscent of those of VEXAS syndrome but without mutation of the UBA1 gene.
Case/discussion: Two male patients consulted for general signs, dermatological symptoms, arthralgia, chondritis and venous thrombosis, like patients in the French cohort suffering from VEXAS syndrome.
Acquired autoinflammatory disorders: A dermatologist's perspective.
Clin Exp Dermatol
December 2024
Department of Dermatology, Venereology and Leprology; Postgraduate Institute of Medical Education and Research; Chandigarh, India.
Autoinflammatory disorders are characterized by dysregulated and disproportionately heightened response of innate immune system (IIS) to molecular patterns associated with damage and pathogens/microbes (DAMPs/PAMPs) with crucial role played by neutrophils and macrophages in disease pathogenesis. They closely resemble connective tissue diseases (CTDs). However, anti-nuclear antibodies (ANA), typically considered a marker of CTDs, are negative in autoinflammatory disorders.
View Article and Find Full Text PDFNonischemic Cardiac Manifestations in VEXAS Syndrome.
JAMA Netw Open
December 2024
Department of Internal Medicine, CEREMAIA, Sorbonne Université, Hôpital Tenon, Assistance Publique-Hôpitaux Paris, Paris, France.
Non-hematological triggers of VEXAS syndrome: A case report and literature review.
Curr Res Transl Med
November 2024
CHU Dupuytren 2, service de rhumatologie, 16, rue B.-Descottes, 87042 Limoges cedex, France.
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