What are the treatment options for Kimura disease?

Management of Kimura Disease

Surgical resection combined with postoperative radiotherapy is the most effective treatment for Kimura disease, with significantly lower recurrence rates compared to surgery or radiotherapy alone. 1

Initial Diagnostic Confirmation

• Obtain tissue diagnosis through incisional or excisional biopsy of the subcutaneous nodules or lymph nodes, as histopathological analysis is the primary diagnostic method for Kimura disease 2
• Document characteristic features including subcutaneous tumor-like nodules in the head and neck region, peripheral eosinophilia, and elevated serum IgE levels 3
• Screen for renal involvement with urinalysis, 24-hour urine protein, serum albumin, and renal function tests, as nephrotic syndrome occurs in 12-16% of cases 3
• If proteinuria is present, perform renal biopsy to assess for focal segmental glomerulosclerosis 3

Treatment Algorithm Based on Disease Severity

For Localized Disease Without Renal Involvement

Primary recommendation: Surgical resection combined with low-dose postoperative radiotherapy 1

• Meta-analysis of 570 patients demonstrates this combination reduces local recurrence risk by 63% compared to surgery alone (RR = 4.72; 95% CI, 2.53-8.82) and by 73% compared to radiotherapy alone (RR = 2.72; 95% CI, 1.47-5.04) 1
• Complete surgical excision should target all visible disease with clear margins 1
• Postoperative radiotherapy should be administered at low doses following surgical healing 1

Alternative for patients refusing surgery or with unresectable disease:

• Radiotherapy alone, though associated with higher recurrence rates than combined therapy 1

For Disease With Renal Involvement (Nephrotic Syndrome)

Initiate methylprednisolone therapy 3

• Start with high-dose methylprednisolone during acute phase until proteinuria resolves and serum albumin normalizes 3
• Transition to long-term maintenance therapy with methylprednisolone 8 mg/day to prevent relapses, as demonstrated effective in preventing recurrence over one-year follow-up 3
• Monitor 24-hour urine protein, serum albumin, and renal function regularly 3

For Steroid-Dependent or Steroid-Refractory Disease

First-line steroid-sparing agent: Dupilumab 4, 5

• Administer loading dose of 600 mg subcutaneously, followed by 300 mg every 2 weeks 4, 5
• Dupilumab demonstrates superior efficacy independent of IgE levels, targeting the Th2 pathway (IL-4/IL-13) directly 4
• Expect reduction in lymph node size within 3 months and sustained control of eosinophil counts even after steroid discontinuation 5
• Particularly valuable in pediatric patients to avoid long-term steroid developmental concerns 5

Second-line option: Mycophenolate mofetil for maintenance therapy 2

• Consider for patients requiring long-term immunosuppression who cannot tolerate or fail dupilumab 2

Omalizumab (anti-IgE) is NOT recommended 4

• Case report demonstrates lack of efficacy after 19 cycles of treatment at 450 mg every 4 weeks, with no reduction in mass size, serum IgE, or eosinophil counts 4

Common Pitfalls to Avoid

• Do not rely on oral corticosteroids alone for long-term management without maintenance therapy, as recurrence is common when dosage is tapered 3, 4
• Do not perform surgical excision alone without considering postoperative radiotherapy, as this results in significantly higher recurrence rates 1
• Do not assume elevated IgE is the primary therapeutic target, as dupilumab's efficacy is independent of IgE reduction, suggesting IL-4/IL-13 pathway is more critical 4
• Do not overlook renal screening at initial diagnosis and during follow-up, as nephrotic syndrome can develop and requires specific management 3

Monitoring During Treatment

• Track lymph node size through physical examination and imaging (ultrasound or CT) every 3 months initially 5
• Monitor peripheral eosinophil counts and serum IgE levels, though IgE may remain elevated despite clinical improvement 4
• For patients with renal involvement, check urinalysis, 24-hour urine protein, and serum albumin every 1-2 months 3
• Assess for dupilumab-related adverse effects, particularly conjunctivitis, though this is uncommon 5