Treatment of Kimura's disease with cyclosporine.
ABSTRACT Kimura's disease is a rare, chronic inflammatory disease of unknown cause. It is characterized by painless subcutaneous swellings and lymphadenopathy, commonly affecting the head and neck region. This is associated with peripheral blood eosinophilia and raised serum IgE. It has distinct histological features of lymphoid follicles, eosinophilic infiltrate, fibrosis and vascular proliferation. The disease usually has a benign, indolent course. Traditionally, therapeutic options have included surgery, radiotherapy and steroids but response has been less than satisfactory. Recently, cyclosporine has been reported to be effective in the treatment of Kimura's disease. In this article, we present a middle-aged Chinese female with Kimura's disease for 20 years and her favourable response to cyclosporine.
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ABSTRACT: Here we report two paediatric cases of Kimura's disease, which is a rare, chronic inflammatory disorder with undetermined aetiology. The first patient was a 17-year-old boy presenting with a swelling behind his right ear and a nephrotic syndrome. The second patient was a 9-year-old boy presenting with a left-sided preauricular swelling. Both displayed peripheral blood eosinophilia, raised levels of serum IgE and typical histological findings. Initial response to prednisolone was excellent but both relapsed as medication was tapered or discontinued. Adding cyclosporine to the regimen resulted in prolonged remission in both patients. CONCLUSION: We support the use of cyclosporine for maintaining remission in paediatric patients with Kimura's disease.Acta Paediatrica 03/2011; 100(10):e186-9. · 1.97 Impact Factor
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