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Kimura disease: a case report and review of the literature with a new management protocol

Mohamed Ashraf Fouda1, Osama Gheith, Ayman Refaie

  • 1Mansoura Urology and Nephrology Center, Mansoura University, Mansoura, Egypt.

International Journal of Nephrology
|March 23, 2011
PubMed

Insights

Kimura disease, a rare inflammatory condition, can present atypically in Caucasian men with nephrotic syndrome. Early diagnosis and treatment with steroids, endoxan, and MMF are crucial for successful outcomes.

Area of Science:

  • Nephrology
  • Immunology
  • Pathology

Background:

  • Kimura disease (KD) is a rare chronic inflammatory disorder characterized by angiolymphatic proliferation.
  • KD typically affects young Asian men but is uncommon in other demographics.
  • Nephrotic syndrome is a frequent comorbidity of Kimura disease.

Observation:

  • This report details an atypical case of Kimura disease in a middle-aged Caucasian male.
  • The patient presented with steroid-responsive early membranous glomerulonephritis.
  • This presentation deviates from the typical demographic and clinical profile of KD.

Findings:

  • Kimura disease can manifest atypically in Caucasian individuals, presenting as secondary steroid-responsive nephrotic syndrome.
  • Treatment with corticosteroids, cyclophosphamide (endoxan), and mycophenolate mofetil (MMF) demonstrated safety and efficacy.
  • Diagnostic challenges exist due to the rarity and atypical presentations of KD, potentially leading to delayed or incorrect evaluations.

Implications:

  • Highlights the importance of considering Kimura disease in Caucasian patients with unexplained nephrotic syndrome.
  • Suggests that established immunosuppressive therapies are effective for atypical KD presentations.
  • Emphasizes the need for increased awareness among clinicians to avoid diagnostic delays and unnecessary procedures for Kimura disease.

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