Advertisement

Clinical Rheumatology

, Volume 30, Issue 6, pp 859–865 | Cite as

Effective treatment of Kimura’s disease with leflunomide in combination with glucocorticoids

  • Lie DaiEmail author
  • Xiu-Ning Wei
  • Dong-Hui Zheng
  • Ying-Qian Mo
  • Frank Pessler
  • Bai-Yu Zhang
Case Based Review

Abstract

Kimura’s disease (KD) is a rare, benign, chronic inflammatory disease which typically presents as persisting or recurring tumor-like lesions in the head and neck area that can be easily misdiagnosed. We report one patient with KD treated with leflunomide in combination with glucocorticoids and analyzed the literature on treatment of KD. The patient had a recurrent mass in the left upper arm with eosinophilia and elevated serum IgE but no renal involvement. The clinical manifestations improved markedly within 1 month, and blood eosinophil count and serum IgE normalized. Corticosteroids were then tapered gradually without recurrence or severe side effects in the 2-year follow-up period. Literature analysis identified four different non-drug interventions and 18 different drugs for treating KD, most of which were obtained from case reports. Our use of combination therapy of leflunomide and glucocorticoids suggests the need for a controlled trial for the treatment of this rare disorder.

Keywords

Eosinophilia Kimura’s disease Leflunomide Lymphadenopathy 

Notes

Acknowledgments

We gratefully thank the cooperation of the KD patient reported here, as well as the members of the medical staff and pathologist who generously collaborated with this study.

Disclosures

None

References

  1. 1.
    Rajpoot DK, Pahl M, Clark J (2000) Nephrotic syndrome associated with Kimura disease. Pediatr Nephrol 14:486–488PubMedCrossRefGoogle Scholar
  2. 2.
    Chang AR, Kim K, Kim HJ et al (2006) Outcomes of Kimura's disease after radiotherapy or nonradiotherapeutic treatment modalities. Int J Radiat Oncol Biol Phys 65:1233–1239PubMedCrossRefGoogle Scholar
  3. 3.
    Hareyama M, Oouchi A, Nagakura H et al (1998) Radiotherapy for Kimura's disease: the optimum dosage. Int J Radiat Oncol Biol Phys 40:647–651PubMedCrossRefGoogle Scholar
  4. 4.
    Kim GE, Kim WC, Yang WI et al (1997) Radiation treatment in patients with recurrent Kimura's disease. Int J Radiat Oncol Biol Phys 38:607–612PubMedCrossRefGoogle Scholar
  5. 5.
    Chen H, Thompson LD, Aguilera NS et al (2004) Kimura disease: a clinicopathologic study of 21 cases. Am J Surg Pathol 28:505–513PubMedCrossRefGoogle Scholar
  6. 6.
    Wang YS, Tay YK, Tan E et al (2005) Treatment of Kimura's disease with cyclosporine. J Dermatolog Treat 16:242–244PubMedCrossRefGoogle Scholar
  7. 7.
    Ben-Chetrit E, Amir G, Shalit M (2005) Cetirizine: an effective agent in Kimura's disease. Arthritis Rheum 53:117–118PubMedCrossRefGoogle Scholar
  8. 8.
    Choi WJ, Hur J, Ko JY, Yeo KY, Kim JS, Yu HJ (2010) An unusual clinical presentation of Kimura's disease occurring on the buttock of a five-year-old boy. Ann Dermatol 22:57–60PubMedCrossRefGoogle Scholar
  9. 9.
    Dik VK, van de Wiel BA, Vasmel WL (2010) Kimura's disease of the parotid glands and multiple cervical lymph nodes. Neth J Med 68:175–177PubMedGoogle Scholar
  10. 10.
    Park JS, Jin W, Ryu KN, Won KY (2009) Bilateral asymmetric superficial soft tissue masses with extensive involvement of both upper extremities: demonstration of Kimura's disease by US and MRI (2008: 12b). Eur Radiol 19:781–786PubMedCrossRefGoogle Scholar
  11. 11.
    Tseng CF, Lin HC, Huang SC, Su CY (2005) Kimura's disease presenting as bilateral parotid masses. Eur Arch Otorhinolaryngol 262:8–10PubMedCrossRefGoogle Scholar
  12. 12.
    Chitapanarux I, Ya-In C, Kittichest R et al (2007) Radiotherapy in Kimura's disease: a report of eight cases. J Med Assoc Thai 90:1001–1005PubMedGoogle Scholar
  13. 13.
    Choi JE, Seo SH, Kim IH, Kye YC, Son SW (2008) Successful treatment of Kimura's disease with a 595-nm ultra-long pulsed dye laser. Acta Derm Venereol 88:315–316PubMedGoogle Scholar
  14. 14.
    Cho MS, Kim ES, Kim HJ, Yang WI (1997) Kimura's disease of the epiglottis. Histopathology 30:592–594PubMedCrossRefGoogle Scholar
  15. 15.
    Tsukagoshi H, Nagashima M, Horie T et al (1998) Kimura's disease associated with bronchial asthma presenting eosinophilia and hyperimmunoglobulinemia E which were attenuated by suplatast tosilate (IPD-1151T). Intern Med 37:1064–1067PubMedCrossRefGoogle Scholar
  16. 16.
    Ohtsuka Y, Shimizu T, Fujii T et al (2004) Pranlukast regulates tumour growth by attenuating IL-4 production in Kimura disease. Eur J Pediatr 163:416–417PubMedGoogle Scholar
  17. 17.
    Hongcharu W, Baldassano M, Taylor CR (2000) Kimura's disease with oral ulcers: response to pentoxifylline. J Am Acad Dermatol 43:905–907PubMedCrossRefGoogle Scholar
  18. 18.
    Kanekura T, Usuki K, Kanzaki T et al (2004) Skin disorders with prominent eosinophilic infiltration treated successfully with nicotine. Report of two cases. Dermatology 208:153–157PubMedCrossRefGoogle Scholar
  19. 19.
    Lee MW, Bae JY, Choi JH, Moon KC, Koh JK (2004) Cutaneous eosinophilic vasculitis in a patient with Kimura's disease. J Dermatol 31:139–141PubMedGoogle Scholar
  20. 20.
    Kabashima R, Kabashima K, Mukumoto S et al (2009) Kimura's disease presenting with a giant suspensory tumor and associated with membranoproliferative glomerulonephritis. Eur J Dermatol 19:626–628PubMedGoogle Scholar
  21. 21.
    Guimaraes CS, Moulton-Levy N, Sapadin A, Vidal C (2009) Kimura's disease. Case Report Med 2009:424053PubMedGoogle Scholar
  22. 22.
    Lenk N, Artüz F, Kulaçoğlu S, Alli N (1997) Kimura's disease. Int J Dermatol 36:437–439PubMedCrossRefGoogle Scholar
  23. 23.
    Obata Y, Furusu A, Nishino T et al (2010) Membranous nephropathy and Kimura's disease manifesting a hip mass. A case report with literature review. Intern Med 49:1405–1409PubMedCrossRefGoogle Scholar
  24. 24.
    Maleki D, Sayyah A, Rahimi-Rad MH, Gholami N (2010) Kimura's disease with eosinophilic panniculitis—treated with cyclosporine: a case report. Allergy Asthma Clin Immunol 6:5PubMedCrossRefGoogle Scholar
  25. 25.
    Natov SN, Strom JA, Ucci A (1998) Relapsing nephrotic syndrome in a patient with Kimura's disease and IgA glomerulonephritis. Nephrol Dial Transplant 13:2358–2363PubMedCrossRefGoogle Scholar
  26. 26.
    Boulanger E, Gachot B, Verkarre V, Valensi F, Brousse N, Hermine O (2002) All-trans-retinoic acid in the treatment of Kimura's disease. Am J Hematol 71:66PubMedCrossRefGoogle Scholar
  27. 27.
    Beccastrini E, Emmi G, Chiodi M, et al (2010) Kimura's disease: case report of an Italian young male and response to oral cyclosporine A in an 8 years follow-up. Clin Rheumatol Apr 19 [Epub ahead of print]Google Scholar
  28. 28.
    Teraki Y, Katsuta M, Shiohara T (2002) Lichen amyloidosus associated with Kimura's disease: successful treatment with cyclosporine. Dermatology 204:133–135PubMedCrossRefGoogle Scholar
  29. 29.
    Vourc'h M, Barbarot S, Stalder JF (2007) Ciclosporin improves quality of life in Kimura's disease. Br J Dermatol 157:420–421PubMedCrossRefGoogle Scholar
  30. 30.
    Wang DY, Mao JH, Zhang Y et al (2008) Kimura disease: a case report and review of the Chinese literature. Nephron Clin Pract 111:c55–c61PubMedCrossRefGoogle Scholar
  31. 31.
    Chartapisak W, Opastirakul S (2002) Steroid-resistant nephrotic syndrome associated with Kimura's disease. Am J Nephrol 22:381–384PubMedCrossRefGoogle Scholar
  32. 32.
    Connelly A, Powell HR, Chan YF, Fuller D, Taylor RG (2005) Vincristine treatment of nephrotic syndrome complicated by Kimura disease. Pediatr Nephrol 20:516–518PubMedCrossRefGoogle Scholar
  33. 33.
    Chung YG, Jee WH, Kang YK et al (2010) Kimura's disease involving a long bone. Skeletal Radiol 39:495–500PubMedCrossRefGoogle Scholar
  34. 34.
    Chong VF, Balakrishnan A, Fong KW (2000) Kimura's disease of the pharynx. Clin Radiol 55:649–651PubMedCrossRefGoogle Scholar
  35. 35.
    Goh Hood Keng C, Pang KP, Teng PW (2006) Kimura's disease of the parapharyngeal space. Ear Nose Throat J 85:106–108PubMedGoogle Scholar
  36. 36.
    Kim CJ, Nam JH, Chung HY et al (2004) Kimura disease in a patient with Rubinstein–Taybi syndrome. Pediatr Int 46:609–611PubMedCrossRefGoogle Scholar
  37. 37.
    Liu C, Hu W, Chen H, Tang Z, Zeng C, Liu Z, Li L (2008) Clinical and pathological study of Kimura's disease with renal involvement. J Nephrol 21:517–525PubMedGoogle Scholar
  38. 38.
    Ghosn S, Bahhady R, Mahfouz R et al (2009) Concomitant occurrence of kimura disease and mycosis fungoides in a Lebanese woman: significance and response to rituximab. Am J Dermatopathol 31:814–818PubMedCrossRefGoogle Scholar
  39. 39.
    Sun QF, Xu DZ, Pan SH et al (2008) Kimura disease: review of the literature. Intern Med J 38:668–672PubMedCrossRefGoogle Scholar
  40. 40.
    Katagiri K, Itami S, Hatano Y et al (1997) In vivo expression of IL-4, IL-5, IL-13 and IFN-gamma mRNAs in peripheral blood mononuclear cells and effect of cyclosporin A in a patient with Kimura's disease. Br J Dermatol 137:972–977PubMedCrossRefGoogle Scholar
  41. 41.
    Kimura Y, Pawankar R, Aoki M et al (2002) Mast cells and T cells in Kimura's disease express increased levels of interleukin-4, interleukin-5, eotaxin and RANTES. Clin Exp Allergy 32:1787–1793PubMedCrossRefGoogle Scholar
  42. 42.
    Lu HJ, Tsai JD, Sheu JC et al (2003) Kimura disease in a patient with renal allograft failure secondary to chronic rejection. Pediatr Nephrol 18:1069–1072PubMedCrossRefGoogle Scholar
  43. 43.
    Maddisom P, Kiely P, Kirkham B et al (2005) Leflunomide in rheumatoid arthritis: recommendations through a process of consensus. Rheumatology (Oxford) 44:280–286CrossRefGoogle Scholar
  44. 44.
    Tam LS, Li EK, Wong CK et al (2006) Safety and efficacy of leflunomide in the treatment of lupus nephritis refractory or intolerant to traditional immunosuppressive therapy :an open label trial. J Ann Rheum Dis 65:417–418CrossRefGoogle Scholar
  45. 45.
    Manna SK, Aggarwal BB (1999) Immunosuppressive leflunomide metabolite (A771726) blocks TNF-dependent nuclear factor-kappa B activation and gene expression. J Immunol 162:2095–2102PubMedGoogle Scholar
  46. 46.
    Siemasko KF, Chong AS, Williams JW et al (1996) Regulation of B cell function by the immunosuppressive agent leflunomide. Transplantation 61:635–642PubMedCrossRefGoogle Scholar
  47. 47.
    Bartlett RR, Mattar T, Weithmann U et al (1989) Leflunomide (HWA 486): a novel immunorestoring drug. In: Therapeutic approaches to inflammatory diseases. Amsterdam, Elsevier North-Holland, pp 215–228Google Scholar
  48. 48.
    Nikcevich DA, Finnegan A, Chong AS-F et al (1994) Inhibition of interleukin 2 (IL-2)-stimulated tyrosine kinase activity by leflunomide. Agents Actions 41:C279–C282CrossRefGoogle Scholar
  49. 49.
    Tsukadaira A, Kitano K, Okubo Y et al (1998) A case of pathophysiologic study in Kimura’s disease: measurement of cytokines and surface analysis of eosinophils. Ann Allergy Asthma Immunol 81:423–427PubMedCrossRefGoogle Scholar
  50. 50.
    Jarman ER, Kuba A, Montermann E et al (1999) Inhibition of murine IgE and immediate cutaneous hypersensitivity responses to ovalbumin by the immunomodulatory agent leflunomide. Clin Exp Immunol 115:221–228PubMedCrossRefGoogle Scholar

Copyright information

© Clinical Rheumatology 2011

Authors and Affiliations

  • Lie Dai
    • 1
    Email author
  • Xiu-Ning Wei
    • 1
  • Dong-Hui Zheng
    • 1
  • Ying-Qian Mo
    • 1
  • Frank Pessler
    • 2
  • Bai-Yu Zhang
    • 1
  1. 1.Department of Rheumatology, Sun Yat-sen Memorial HospitalSun Yat-sen UniversityGuangzhouPeople’s Republic of China
  2. 2.Department of Infection GeneticsHelmholtz Centre for Infection ResearchBraunschweigGermany

Personalised recommendations