Further characterization of clinical and laboratory features in VEXAS syndrome: large-scale analysis of a multicentre case series of 116 French patients.

Background: A new autoinflammatory syndrome related to somatic mutations of UBA1 was recently described and called VEXAS syndrome ('Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome').

Objectives: To describe clinical characteristics, laboratory findings and outcomes of VEXAS syndrome.

Methods: One hundred and sixteen patients with VEXAS syndrome were referred to a French multicentre registry between November 2020 and May 2021.

Primary varicella-zoster virus infection of the immunocompromised associated with acute pancreatitis and hemophagocytic lymphohistiocytosis: A case report.

Rationale: Primary varicella-zoster virus (VZV) infection may be associated with hemophagocytic lymphohistiocytosis (HLH), as well as with acute pancreatitis. However, there is few data concerning the evolution and the optimal treatment of these rare associations.

Patient Concerns: A 57-year-old immunocompromised woman, who was treated for chronic lymphocytic leukemia 3 years prior to admission, was hospitalized with abdominal pain revealing severe acute pancreatitis.

[Libman-Sacks endocarditis under apixaban in a patient with a high-risk profile venous antiphospholipid syndrome].

Introduction: Libman-Sacks endocarditis is a rare complication of antiphospholipid syndrome. Anti-vitamin K therapy is the standard treatment, although valvular replacement surgery may be required in some severe cases. In the latest EULAR recommendations, it is advised not to use direct oral anticoagulants in the management of antiphospholipid syndrome, especially of high-risk profile.