Abstract

In the realm of modern medicine, the discovery of novel diseases often presents both challenges and opportunities. VEXAS syndrome, an acronym for Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic syndrome, is a striking example of a unique monogenic disorder that has recently come to light. This syndrome represents a bridge between the worlds of rheumatology and hematology, and its clinical manifestations have left clinicians and researchers perplexed. In this editorial, I will delve into the complexity of VEXAS syndrome, exploring its clinical features, genetic underpinnings, and the quest for effective treatments. Furthermore, I will discuss how VEXAS syndrome may serve as a prototype for a new class of diseases that blur the boundaries between hematopoiesis and inflammation.

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