Kimura Disease Treatment & Management

Updated: Mar 15, 2022
  • Author: Alan Snyder; Chief Editor: William D James, MD  more...
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Treatment

Medical Care

Observation is acceptable if the Kimura disease lesions are neither symptomatic nor disfiguring.

Oral corticosteroids are commonly used; however, the disease frequently recurs after cessation of therapy. Intralesional corticosteroids may be effective for localized disease. [17]

Oral corticosteroids in combination with cetirizine may prove to be an effective alternative treatment to surgery to reduce related nodular masses. In one patient, continued daily cetirizine prevented recurrence 4 months after tapering steroids. [33]

Leflunomide in combination with oral prednisone may be an option for treating patients with or without renal involvement who are unresponsive to corticosteroids alone. The subject of 1 study remained disease free at the 12-month follow up after treatment with leflunomide and methylprednisone. [48] Leflunomide may have an antiproliferative effect on eosinophils.

Cyclosporine has been reported to induce remission in patients with Kimura disease. [49, 50, 51] A dose of 5 mg/kg/d was effective, but the lesions may recur upon cessation of therapy. [52]

Intravenous immunoglobulin (IVIG) was used in 1 patient as a steroid-sparing agent, and he remained disease-free more than 6 years after follow-up. [53]

Oral pentoxifylline has been reported to be effective in 1 patient with Kimura disease; however, the lesions relapsed after discontinuation of therapy. [54]

Dupilumab has been reported to be effective in 1 patient with Kimura disease after surgical excision. The patient was started on dupilumab 600 mg followed by 300 mg every 2 weeks for 8 months. No recurrence was seen in 1 year of followup. [55]

All trans-retinoic acid in combination with prednisone has resulted in remission of Kimura disease in 1 patient, and he remained disease free 12 months after discontinuation of all therapy. [56]

Imatinib may be an effective treatment for Kimura disease, based on advances in research for therapy in hypereosinophilic syndrome, but further investigation is necessary. [19]

Photodynamic therapy has been used successfully in 1 patient who experienced recurrence of disease after initial surgical management. [13]

Radiotherapy has occasionally been used to treat recurrent or persistent Kimura disease lesions. A report by Hareyama et al [57] described the use of radiotherapy at dosages of 26-30 Gy; local control was achieved in 74% of lesions. Another study demonstrated that radiotherapy (20-45 Gy) was more effective than local excision and steroid treatment, with local response rates of 64.3% versus 22.2%, respectively. No adverse effects were observed during a mean follow-up period of 65 months. [58] New technology such as 3-dimensional printing is being explored for preventing collateral damage during radiotherapy bolus delivery to radiosensitive areas such as the head and neck. [59] However, considering the benign nature of Kimura disease, radiation should be reserved for recurrent or disfiguring lesions.

The largest retrospective meta-analysis of Kimura disease treatment to date (n=639) concluded that surgical resection with low-dose postoperative radiotherapy resulted in the lowest recurrence rate of Kimura disease. Furthermore, it suggested that corticosteroid therapy should be a second-line option for treatment, owing to the potential adverse effects of long-term corticosteroid use and the high rates of recurrence when used alone. [60] The same conclusions were made in another study comparing recurrence rates in 46 patients who underwent steroid therapy, surgical excision, radiotherapy, or surgical excision with radiotherapy. [17]