Abstract
Kimura's disease (KD) is a rare chronic inflammatory disorder of unknown etiology that manifests as painless subcutaneous lesions in the head and neck. It primarily affects young Asian males and is characterized by peripheral eosinophilia and elevated levels of serum Immunoglobulin (Ig)E. Histologically, a variety of renal lesions have been observed in patients with KD, although IgA nephropathy is rarely reported. In the current study, a case of KD with an atypical manifestation accompanied with IgA nephropathy was reported in a middle-aged Chinese man. The subcutaneous lesion was located on the elbow of the patient. Although the recurrence rate of KD-associated nephrotic syndrome is high, the patient has not experienced relapse following 6 months of pulse treatment with methylprednisone and cyclophosphamide. In conclusion, methylprednisone combined with cyclophosphamide is effective in the treatment of KD-associated IgA nephropathy, and long-term administration of methylprednisone may achieve the effect of preventing KD recurrence.
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