Autoinflammatory diseases induce recurrent systemic inflammatory attacks—often accompanied by fever—that occur independently of infectious triggers [1]. In daily practice, a major challenge arises when patients display a convincing autoinflammatory phenotype with objective inflammation, yet extensive next-generation sequencing (NGS) investigations remain non-diagnostic. In such settings, clinicians are left with marked clinical heterogeneity, no universally accepted diagnostic criteria, and largely empirical, attack-driven treatment decisions.
