A case study of Kimura’s Disease in a 14-Year-Old Boy with Nephrotic Syndrome

A 14-year-old boy presented with generalized edema and reduced urine output for 15 days, accompanied by a painless swelling in the left parotid region that had developed over two years. Clinical examination revealed pallor, generalized lymphadenopathy, and a notable parotid swelling measuring 6×5 cm. Laboratory tests indicated 20% eosinophils with an absolute eosinophil count (AEC) of 2240/µL, nephrotic-range proteinuria (UPCR 15.9), hypoalbuminemia (1.08 g/dl), normal renal function, and significantly elevated serum immunoglobulin E (IgE) levels exceeding 2500 IU/ml.

Investigations for infections, malignancies, and autoimmune disorders returned negative results. An ultrasound identified a hypoechoic lesion in the left parotid region. Differential diagnoses included lymphoma and Langerhans cell histiocytosis with secondary nephrotic syndrome. A renal biopsy confirmed focal segmental glomerulosclerosis (FSGS, tip lesion), while excision biopsies of the parotid and lymph nodes showed histopathological features consistent with Kimura’s disease (KD).

Treatment involved local excision, corticosteroids, and cyclosporine (2 mg/kg/d), resulting in improvements in edema, lymphadenopathy, and a reduction in proteinuria within six weeks. Corticosteroids were gradually tapered and discontinued after 12 weeks, while cyclosporine was continued. At the 12-week follow-up, the patient was asymptomatic, with no proteinuria and a normal eosinophil count.

Therefore, KD is a rare, chronic benign inflammatory condition primarily affecting Southeast Asian middle-aged males, with few cases in children. It is characterized by painless subcutaneous masses, eosinophilia, and elevated IgE levels, with kidney involvement being a rare systemic manifestation.

Source: Jayachandran M, B.C. R, Choudhary A. et al. FSGS in Kimura Disease - A Rare Cause of Nephrotic Syndrome in an Adolescent. Indian J Pediatr.2025. https://doi.org/10.1007/s12098-025-05509-7

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