Chronic urticaria and autoinflammatory syndromes

Purpose of review This review provides an updated overview of the association between chronic urticaria (CU) and autoinflammatory syndromes (AS), underlining the diagnostic and therapeutic implications of identifying CU as an initial manifestation of systemic autoinflammatory disorders. Recent findings emerging evidence has reinforced the role of innate immune dysregulation in the pathogenesis of CU associated with AS, with particular involvement of the pro-inflammatory cytokines such as interleukin (IL)-1β. Several monogenic and multifactorial autoinflammatory diseases, including cryopyrin-associated periodic syndromes (CAPS), Schnitzler syndrome (SchS), Still's disease (SD), and others, may present with CU. Neutrophilic urticarial dermatosis (NUD) has been recognized as a histopathological hallmark. Early diagnosis remains challenging but is crucial, as targeted therapies, especially IL-1 inhibitors, have demonstrated significant efficacy in controlling systemic inflammation and preventing disease progression. Summary CU refractory to conventional treatment, particularly when associated with systemic symptoms, should prompt suspicion of an underlying autoinflammatory syndrome. A comprehensive diagnostic approach, including clinical assessment, inflammatory markers evaluation, histopathological examination, and genetic testing, is essential. Recognition of the autoinflammatory nature of CU allows for timely initiation of personalized therapies, improving patient prognosis and reducing long-term morbidity.