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The clinicopathological characteristics of Kimura disease in Chinese patients

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Abstract

Introduction

Kimura disease (KD) is a rare idiopathic inflammatory disorder of unknown etiology. Unusual presentations of KD might cause diagnostic difficulty or be misdiagnosed as malignancy if clinical suspicion is insufficiently high. Here, we aimed to determine the clinicopathological features of Chinese KD patients to reveal further insights into the natural history and treatment of this disease.

Method

The clinical data of 46 cases of KD diagnosed at Peking Union Medical College Hospital from January 1980 to December 2018 were analyzed retrospectively through case record review.

Results

Of 46 cases, 40 were male and six were female. The age at onset ranged from 2 to 56 years (median 27 years). All patients presented with either single (26.1%) or multi-focal (73.9%) subcutaneous masses. Twenty-nine (63.0%) cases presented with head and neck subcutaneous masses, and 9 cases (19.6%) involved different parts of the body. Parotid, submandibular, and lacrimal gland involvement occurred in 17 (37.0%), 3 (6.5%), and 2 cases (4.3%), respectively. Nephrotic syndrome was present in three cases (6.5%), and thromboembolism was present in five cases (10.9%). During follow-up, thirteen patients (13/28, 46.4%) relapsed over 1–13 years (median 8.5 years). The recurrence rate in patients receiving corticosteroids, surgery, and combined surgery and radiotherapy was 30.8%, 66.7%, and 50.0%, respectively. One patient was diagnosed with T cell lymphoma 1 year after diagnosis of KD.

Conclusions

KD is characterized by subcutaneous masses but it is also a systemic disease. Given the high rate of recurrence and reported association with lymphoma, patients require careful long-term follow-up.

Key Points

• Kimura disease (KD) is a rare inflammatory disorder of unknown etiology that is endemic in Asia.

• Clinicians must regard and manage KD as a systemic disease.

• There is no consensus on optimal treatments and further studies are necessary to improve outcomes.

• Given the high rate of recurrence and reported association with lymphoma, patients require careful long-term follow-up.

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References

  1. Li T, Chen X, Wang S et al (1996) A clinicopathologic study of 54 Chinese patients. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 82:549–555

    Article  CAS  Google Scholar 

  2. Gao Y, Chen Y, Yu G et al (2006) Clinicopathologic study of parotid involvement in 21 cases of eosinophilic hyperplastic lymphogranuloma (Kimura’s disease). Oral Surg Oral Med Oral Pathol Oral Radiol Endod 102:651–658

    Article  Google Scholar 

  3. Kottler D, Barete S, Quereux G et al (2015) Retrospective multicentric study of 25 Kimura disease patients: emphasis on therapeutics and shared features with cutaneous IgG4-related disease. Dermatology 231:367–377

    Article  CAS  Google Scholar 

  4. Abuel-Haija M, Hurford MT (2007) Kimura disease. Arch Pathol Lab Med 131:650–651

    PubMed  Google Scholar 

  5. Jin X, Shi T (1937) Eosinophilic lymphoblastoma: a report of 7 cases similar to Mikulicz’s disease. Zhonghua Yixue Zazhi (in Chinese) 23:681–699

    Google Scholar 

  6. Kimura T, Yoshimura S, Ishikawa E (1948) On the unusual granulation combined with hyperplastic changes of lymphatic tissues. Trans Soc Pathol Jpn 37:179–180

  7. Sun QF, Xu DZ, Pan SH, Ding JG, Xue ZQ, Miao CS, Cao GJ, Jin DJ (2008) Kimura disease: review of the literature. Intern Med J 38(8):668–672

    Article  CAS  Google Scholar 

  8. Fouda MA, Gheith O, Refaie A, El-Saeed M, Bakr A, Wafa E, Abdelraheem M, Sobh M (2011) Kimura disease: a case report and review of the literature with a new management protocol. Int J Nephrol 2010:673908

    PubMed  PubMed Central  Google Scholar 

  9. Wang D, Mao JH, Zhang Y, Gu WZ, Zhao SA, Chen YF, Liu AM (2009) Kimura disease: a case report and review of the Chinese literature. Nephron Clin Pract 111:c55–c61

    Article  CAS  Google Scholar 

  10. Ren S, Li XY, Wang F, Zhang P, Zhang Y, Li GS, Wang L, Zhong X (2018) Nephrotic syndrome associated with Kimura’s disease: a case report and literature review. BMC Nephrol 19:316

    Article  CAS  Google Scholar 

  11. Obata Y, Furusu A, Nishino T, Ichinose H, Ohnita A, Iwasaki K, Taguchi T, Kohno S (2010) Membranous nephropathy and Kimura’s disease manifesting a hip mass. A case report with literature review. Intern Med 49:1405–1409

    Article  Google Scholar 

  12. Heo W, Jun HJ, Kang DK, Min HK, Hwang YH, Kim JY, Nam K (2017) Acute limb ischemia and coronary artery disease in a case of Kimura’s disease. Korean J Thorac Cardiovasc Surg 50:114–118

    Article  Google Scholar 

  13. Nagashima T, Kamimura T, Nara H, Iwamoto M, Okazaki H, Minota S (2006) Kimura’s disease presenting as steroid-responsive thromboangiitis obliterans. Circulation 114:e10–e11

    Article  Google Scholar 

  14. Danis R, Ozmen S, Akin D, Ozekinci S, Altintas A, Cil T, Pasa S, Kihnc I (2009) Thrombosis of temporal artery and renal vein in Kimura disease related nephrotic syndrome. J Thromb Thrombolysis 27:115–118

    Article  Google Scholar 

  15. Eguia B, Bachmeyer C, Charlotte F, Cabannes-Hamy A, Loustau V, Senet P, Davi F, Frances C (2011) Kimura disease with necrosis of the limbs and mononeuritis multiplex. Clin Exp Dermatol 36:329–331

    Article  CAS  Google Scholar 

  16. Lee J, Hong YS (2014) Kimura disease complicated with bowel infarction and multiple arterial thrombosis in the extremities. J Clin Rheumatol 20:38–41

    Article  Google Scholar 

  17. Liu H, Al-Quran SZ, Lottenberg R (2010) Thrombotic storm in Kimura disease. J Thromb Thrombolysis 29:354–357

    Article  Google Scholar 

  18. Terriers B, Piette AM, Kerob D et al (2006) Superficial venous thrombophlebitis as the initial manifestation of hypereosinophilic syndrome: study of the first 3 cases. Arch Dermatol 142:1606–1610

    Google Scholar 

  19. Kanno H, Ouchi N, Sato M, Wada T, Sawai T (2005) Hypereosinophilia with systemic thrombophlebitis. Hum Pathol 36:585–589

    Article  Google Scholar 

  20. Chen H, Thompson LD, Aguilera NS et al (2004) Kimura disease: a clinicopathologic study of 21 cases. Am J Surg Pathol 28:505–513

    Article  Google Scholar 

  21. Tham KT, Leung PC, Saw D, Gwi E (1981) Kimura’s disease with salivary gland involvement. Br J Surg 68:495–497

    Article  CAS  Google Scholar 

  22. Hattori T, Miyanaga T, Tago O et al (2012) Isolated cutaneous manifestation of IgG4-related disease. J Clin Pathol 65:815–818

    Article  Google Scholar 

  23. McKelvie PA, Lyons B, Barnett G, Allen PW (2012) Kimura’s disease in two Caucasians, one with multiple recurrences associated with prominent IgG4 production. Pathology 44:275–278

    Article  Google Scholar 

  24. Liu L, Chen Y, Fang Z, Kong J, Wu X, Zhang Z (2015) Kimura’s disease or IgG4-related disease? A case-based review. Clin Rheumatol 34:385–389

    Article  Google Scholar 

  25. Li J, Ge X, Ma J, Li M, Li J (2014) Kimura’s disease of the lacrimal gland mimicking IgG4-related orbital disease. BMC Ophthalmol 14:158

    Article  CAS  Google Scholar 

  26. Lee JH, Kim JH, Lee SU, Kim SC (2018) Orbital mass with features of both Kimura disease and immunoglobulin G4-related disease. Ophthal Plast Reconstr Surg 34:e121–e123

    PubMed  Google Scholar 

  27. Stone JH, Zen Y, Deshpande V (2012) IgG4-related disease. N Engl J Med 366:539–551

    Article  CAS  Google Scholar 

  28. Tsubouchi K, Imanaga T, Yamamoto M, Hirata K, Nakano T (2010) A case of IgG4-positive multi-organ lymphoproliferative syndrome associated with Kimura disease (in Japanese). Nihon Kokyuki Gakkai Zasshi 48:524–528

  29. Ye P, Wei T, Yu GY, Wu LL, Peng X (2016) Comparison of local recurrence rate of three treatment modalities for Kimura disease. J Craniofac Surg 27:170–174

    Article  Google Scholar 

  30. Ye P, Ma DQ, Yu GY, Gao Y, Peng X (2017) Comparison of the efficacy of different treatment modalities for Kimura’s disease. Int J Oral Maxillofac Surg 46:350–354

    Article  CAS  Google Scholar 

  31. Kapoor NS, O’Neill JP, Katabi N, Wong RJ, Shah JP (2012) Kimura disease: diagnostic challenges and clinical management. Am J Otolaryngol 33:259–262

    Article  Google Scholar 

  32. Chen HS, Chen H, Zhang NX, Yang QY, Fang LH, Sun FJ, Liu EB, Ji HA (2007) Kimura disease transformed to cutaneous T-cell lymphoma after 38 years: a case report (in Chinese). Chin J Hematol 28:271–272

    CAS  Google Scholar 

  33. Chen XY, Cai JF, Pan HH, Zheng QK (2005) Kimura disease evolved into non-Hodgkin’s lymphoma: a case report (in Chinese). Chin J Hematol 26:615–615

    Google Scholar 

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Authors and Affiliations

  1. Department of Health Care, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

    Xuehan Zhang

  2. Department of General Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1, Shuaifuyuan, Wangfujing St., Beijing, 100730, China

    Yang Jiao

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  1. Xuehan Zhang
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Correspondence to Yang Jiao.

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Ethics approval

This study protocol has been approved by the Ethics Committee of Peking Union Medical College Hospital (protocol number: S-K 702). The ethics committee waived the requirement for informed consent because anonymous data were analyzed retrospectively.

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Zhang, X., Jiao, Y. The clinicopathological characteristics of Kimura disease in Chinese patients. Clin Rheumatol 38, 3661–3667 (2019). https://doi.org/10.1007/s10067-019-04752-6

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  • DOI: https://doi.org/10.1007/s10067-019-04752-6

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