VEXAS and Sweet's Syndrome

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Sweet's syndrome can be a symptom of VEXAS syndrome in men.

VEXAS, DermNet NZ dermnetnz.org/topics/vexas-...

"VEXAS syndrome typically responds poorly to treatment, with high dose systemic corticosteroids (> 20mg/d) showing the most consistent benefit for the inflammatory features. Other DMARDs and immunosuppressive drugs are ineffective. Colchicine may reduce the steroid requirement. Tocilizumab is a theoretical option being trialled. "

CASE-STUDY. ncbi.nlm.nih.gov/pmc/articl...

Steroid reduction‐resistant pulmonary involvement with Sweet's syndrome suspected of being vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic syndrome: A case report, Feb 2024.

- VEXAS syndrome is almost exclusively described in males. One case in a female with a single X-chromosome.

- Diagnosis is currently based on the presence of a positive UBA1 gene variant.

- VEXAS can affect the lungs.

"In a CT review of 45 cases of VEXAS syndrome with pulmonary involvement by Borie et al., bronchopneumonia‐like and organizing pneumonia‐like, as well as heart failure‐like shadows with unilateral pleural effusion and lymphadenopathy were reported."

- High dose steroids used to reduce inflammation. Other immunosuppressants are ineffective.

- Janus kinase inhibitors, e.g. ruxolitinib, can be used to manage symptoms when steroids are reduced.

- In male patients with Sweet's syndrome where there is lung involvement, and where their condition keeps flaring up when steroids are reduced, VEXAS should be considered.