Kimura Disease and Associated Chronic Inflammatory Conditions
Summary
Kimura disease is an uncommon lymphoproliferative fibroinflammatory disorder that predominantly affects young men of Asian descent, manifesting most often as painless subcutaneous nodules in the head and neck region. The hallmark features include marked eosinophilia and elevated serum immunoglobulin E, reflecting a skewed T helper type 2 cytokine milieu. Histopathology reveals florid follicular hyperplasia with germinal centre expansion, prominent post-capillary venule proliferation and dense tissue eosinophilia. Although the aetiology remains obscure, immune dysregulation is implicated, and the condition can be accompanied by renal involvement in the form of nephrotic syndrome. Differentiation from neoplastic and IgG4-related disorders is critical, as clinicopathological overlap may lead to misdiagnosis. Conventional management consists of surgical excision, corticosteroid therapy and, in refractory cases, radiotherapy. Emerging evidence supports the use of targeted biologics to modulate aberrant immune pathways. Globally, heightened awareness of Kimura disease and its chronic inflammatory associations is essential to refine diagnostic pathways, limit unnecessary procedures and develop standardised treatment protocols.
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Kimura Disease and Associated Chronic Inflammatory Conditions publication trend
The graph below shows the total number of articles in kimura disease and associated chronic inflammatory conditions across all publications each year (not limited to Nature Index journals).
Technical terms
Lymphoproliferative disorder: A condition characterised by abnormal proliferation of lymphocytes within lymphoid tissues.
Eosinophilia: An increased number of eosinophils in the blood, often signifying allergic or parasitic processes.
Immunoglobulin E (IgE): An antibody class associated with allergic responses and defence against parasites.
Nephrotic syndrome: A kidney disorder marked by heavy proteinuria, hypoalbuminaemia and peripheral oedema.
IgG4-related disease: A systemic fibroinflammatory condition characterised by IgG4-positive plasma cell infiltration and organ enlargement or fibrosis.
Biologics: Therapeutic agents derived from biological sources that target specific immune molecules or pathways.
References
- Coexisting Nodular Sclerosis Hodgkin Lymphoma and Kimura’s Disease: A Case Report and Literature Review. International Journal of Molecular Sciences (2023).
- Kimura’s disease: A clinicopathological study of 23 cases. Frontiers in Medicine (2022).
- Successful treatment of dupilumab in Kimura disease independent of IgE: A case report with literature review. Frontiers in Immunology (2022).
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