13Oct 2021
Further characterization of clinical and laboratory features occurring in VEXAS syndrome in a large‐scale analysis of multicenter case‐series of 116 French patients

Further characterization of clinical and laboratory features occurring in VEXAS syndrome in a large‐scale analysis of multicenter case‐series of 116 French patients

A new autoinflammatory syndrome related to somatic mutations of UBA1 was recently described and called VEXAS syndrome. To describe clinical characteristics, laboratory findings and outcomes of VEXAS syndrome. Patients referred to a French multicenter registry between November 2020 and May 2021. 116 patients with VEXAS syndrome. Frequency and median of parameters and vital status, from diagnosis to the end of the follow‐up. Main clinical features were skin lesions (83.5%), non‐infectious fever (63.6%), weight loss (62%), lung involvement (49.6%), ocular symptoms (38.8%), relapsing chondritis (36.4%), venous thrombosis (34.7%), lymph nodes (33.9%), and arthralgia (27.3%). Hematological disease was present in 58 cases (50%)...

  • #dermatology

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