Kimura Disease Treatment & Management

Updated: Apr 27, 2017
  • 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. [42]

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. [24]

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 one study remained disease free at the 12-month follow up after treatment with leflunomide and methylprednisone. [43] Leflunomide may have an antiproliferative effect on eosinophils.

Cyclosporine has been reported to induce remission in patients with Kimura disease. [44, 45, 46] A dose of 5 mg/kg/d was effective, but the lesions may recur upon cessation of therapy. [47]

Intravenous immunoglobulin (IVIG) was used in one patient as a steroid-sparing agent, and he remained disease free more than 6 years after follow-up. [48]

Oral pentoxifylline has been reported to be effective in one patient with Kimura disease; however, the lesions relapsed after discontinuation of therapy. [49]

All trans-retinoic acid in combination with prednisone has resulted in remission of Kimura disease in one patient, and he remained disease free 12 months after discontinuation of all therapy. [50]

Imatinib may be an effective treatment for Kimura disease, based on advances in research for therapy in hypereosinophilic syndrome, but further investigation is necessary. [12]

Photodynamic therapy has been used successfully in one patient who experienced recurrence of disease after initial surgical management. [33]

Radiotherapy has occasionally been used to treat recurrent or persistent Kimura disease lesions. A report by Hareyama et al [51] 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. [52] New technology such as three-dimensional printing is being explored for preventing collateral damage during radiotherapy bolus delivery to radiosensitive areas such as the head and neck. [53] 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. [54] 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. [42]

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Surgical Care

Conservative surgical excision has been considered the treatment of choice for Kimura disease. [42, 55] Recurrence after surgery is frequently observed.

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Consultations

Consultation with an otolaryngologist or ophthalmologist should be considered for further evaluation depending on the extent and location of the disease.

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