Kimura’s disease (KD) is a rare, chronic inflammatory disorder that predominantlyaffects young men of East Asian descent. It is characterized by painless solid massesprimarily localized to the deep subcutaneous tissues of the head and neck, eosinophilia,and elevated serum immunoglobulin E (IgE). While the exact cause remains unclear, thepathogenesis is thought to involve dysregulated immune responses, particularly thosemediated by T-helper cells 2 (Th2), eosinophils, and IgE production. Advances in molecularbiology have suggested that genetic factors play a significant role in the developmentand progression of this chronic inflammatory condition. Recent studies have implicatedseveral genes and immune pathways in its development, and understanding these geneticcomponents may provide insights into better diagnostic tools and therapeutic strategies forKD. In this regard, biological therapies, by targeting the immune mechanisms underlyingKD, have been used to treat this challenging condition with promising results, contributingto a better understanding of the pathogenesis of this rare disorder. The aim of this studywas to review the literature concerning the genetic factors and immune mechanisms thatcontribute to the pathogenesis of KD, with a special focus on the role of biological therapies.
Narrative review of genetic and immunological mechanisms involved in the pathogenesis of Kimura’s disease: new therapeutic targets
Cavaliere, Carlo;
2025-01-01
Abstract
Kimura’s disease (KD) is a rare, chronic inflammatory disorder that predominantlyaffects young men of East Asian descent. It is characterized by painless solid massesprimarily localized to the deep subcutaneous tissues of the head and neck, eosinophilia,and elevated serum immunoglobulin E (IgE). While the exact cause remains unclear, thepathogenesis is thought to involve dysregulated immune responses, particularly thosemediated by T-helper cells 2 (Th2), eosinophils, and IgE production. Advances in molecularbiology have suggested that genetic factors play a significant role in the developmentand progression of this chronic inflammatory condition. Recent studies have implicatedseveral genes and immune pathways in its development, and understanding these geneticcomponents may provide insights into better diagnostic tools and therapeutic strategies forKD. In this regard, biological therapies, by targeting the immune mechanisms underlyingKD, have been used to treat this challenging condition with promising results, contributingto a better understanding of the pathogenesis of this rare disorder. The aim of this studywas to review the literature concerning the genetic factors and immune mechanisms thatcontribute to the pathogenesis of KD, with a special focus on the role of biological therapies.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.


