Successful treatment of dupilumab in Kimura disease independent of IgE: A case report with literature review

Abstract

Kimura disease (KD) is a rare and benign chronic inflammatory disease of unknown cause. It is characterized by subcutaneous granuloma of soft tissues in the head and neck region, increased eosinophil count, and elevated serum IgE. Currently, no definitive treatments are recommended. A 57-year-old Chinese man was diagnosed with KD after 7 years of slow subcutaneous masses growth. The patient underwent treatment of oral glucocorticoids for 1 year, but the masses recurred as the dosage was tapered down. Subsequent anti-IgE therapy of omalizumab administered subcutaneously at 450 mg/day at a 4-week interval did not show improvement. The size of masses and serum IgE and circulating eosinophils did not decrease significantly after 19 cycles of continuous treatment. Ultimately, switched strategy of dupilumab was applied at an initial dose of 600 mg, followed by 300 mg every 2 weeks for 4 months. This treatment demonstrated dramatical effects with reduced masses in each area and fast dropdown of eosinophil counts, while the high level of serum IgE remained without changes. Recently, different biologics including anti-IgE, anti-IL-5, and anti-IL-4/IL-13 have been applied to treat KD with satisfied results and help to explore the pathogenesis of this rare disease. To our knowledge, this is the first report that demonstrates the effects of two different biologics in the same patient and reveals the impressive clinical efficacy of dupilumab to treat KD independent of IgE. Therefore, further investigation of the underlying mechanism and the development of diagnosis and treatment of KD is valuable.

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

“…Further reports were found for Kimura’s disease with 4 patients treated with dupilumab and 3/4 experiencing complete and one partial remission [ 232 , 233 , 234 , 235 ]. Four cases of steroid-refractory papuloerythroderma Ofuji treated with dupilumab all reported complete resolution of skin lesions and pruritus [ 236 , 237 , 238 ].…”

Bilateral swelling of the salivary glands and sicca symptoms: an unusual differential diagnosis—Kimura’s disease, a rare allergic condition with a high IgE serum level—a case report and review of the literature

“…In another case, report on a patient with Kimura’s disease oral glucocorticoids had been administered for 1 year, but the masses recurred as the dosage was tapered down. 31 Subsequently, anti-IgE therapy with omalizumab was administered at 450 mg s.c. at 4-week intervals. The size of the masses, the serum IgE level and the percentage of eosinophils did not decrease significantly after 19 cycles of continuous treatment.…”

“…Taken together, the clinical picture of Kimura‘s disease differs and therapy is not straight forward, 33 but there are developments, especially with anti-IgE and anti IL-4/IL-13 antibodies. 31 32 Surgical procedures and radiotherapy have also been successfully used, especially in patients with large masses. 34 However, several reports also stress that the course of the disease may be rather benign—even with no specific therapy.…”

“…In another case, report on a patient with Kimura’s disease oral glucocorticoids had been administered for 1 year, but the masses recurred as the dosage was tapered down 31. Subsequently, anti-IgE therapy with omalizumab was administered at 450 mg s.c. at 4-week intervals.…”

Clinical effects of dupilumab: A novel treatment for Kimura disease

“…However, no pediatric reports or treatments for renal involvement have been published to date (Table 1). [17][18][19][20][21] Our patient presented with persistently high levels of serum IgE despite corticosteroid treatment and rebounded eosinophilia with mild enlargement of the lymph nodes after the corticosteroids were tapered. Thus, we chose dupilumab as a treatment option because it decreases both eosinophil count and serum IgE levels, making it more effective than omalizumab.…”