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Chronic urticaria and autoinflammatory syndromes.

Mona-Rita Yacoub1,2, Arianna Ferlito1, Eustachio Nettis3

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Chronic urticaria (CU) can signal underlying autoinflammatory syndromes (AS). Early diagnosis and targeted therapies, like IL-1 inhibitors, improve outcomes for patients with systemic inflammation.

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Area of Science:

  • Immunology
  • Dermatology
  • Rheumatology

Background:

  • Chronic urticaria (CU) is increasingly recognized as a potential early manifestation of autoinflammatory syndromes (AS).
  • Innate immune dysregulation, particularly involving interleukin-1 beta (IL-1β), plays a key role in the pathogenesis of CU associated with AS.
  • Several autoinflammatory diseases, including cryopyrin-associated periodic syndromes (CAPS), Schnitzler syndrome (SchS), and Still's disease (SD), can present with CU.

Purpose of the Study:

  • To provide an updated overview of the association between chronic urticaria and autoinflammatory syndromes.
  • To highlight the diagnostic and therapeutic implications of recognizing CU as an initial sign of systemic autoinflammatory disorders.

Main Methods:

  • Review of emerging evidence on the link between CU and AS.
  • Analysis of pathogenetic mechanisms involving innate immunity and cytokines.
  • Evaluation of diagnostic approaches and therapeutic strategies, including IL-1 inhibitors.

Main Results:

  • Neutrophilic urticarial dermatosis (NUD) is a recognized histopathological hallmark.
  • Early diagnosis of AS in CU patients is crucial for effective management.
  • Targeted therapies, especially IL-1 inhibitors, show significant efficacy in controlling inflammation and preventing disease progression.

Conclusions:

  • CU refractory to standard treatments, especially with systemic symptoms, warrants investigation for underlying AS.
  • A comprehensive diagnostic workup, including clinical, laboratory, histopathological, and genetic assessments, is essential.
  • Identifying the autoinflammatory nature of CU enables timely, personalized therapy, improving patient prognosis and reducing long-term morbidity.