Kimura’s disease associated with Nephrotic syndrome

Chronic inflammatory condition with tumor like lesions in soft tissue, LN, parotid gland 1st described in China in 1937 Widely recognized by Kimura in Japan in 1948 About 120 cases described, mostly from Asian conturies Kimura’s disease (KD)

Endemic to Asians, male preponderance Benign painless soft tissue swelling around head and neck area ( parotid gland, periauricular, axillary LN ) Lab : pph eosinophilia (98%), elevated serum IgE level Diagnosis : LN biopsy

Lymphoid hyperplasia with germinal centers Eosinophilic and plasma cell infilatration as well as eosinophilic microabscess Various amount of fibrosis and proliferation of postcapillary venules Pathology of Kimura’s disease

Pathogenesis of KD Viral infection or various toxic substances -> alter T cell immunoregulation or induce an IgE mediated type 1 hypersensitivity -> resulting in the release of lymphokine These immunological triggers could result in LN alteration Corticosteroid are mainstay of treatment for KD, suggesting defect in T cell immunoregulation

Common finding of eosinophilia and high serum IgE - strong evidence that relates eosinophilotropic cytokines such as IL4, IL13, to Kimura’s disease Elevated IL4, IL5, IL13 in pts with Kimura’s disease Th2 cell plays the major role in production of cytokines, including IL4, IL13, both of which regulate IgE and IL5

Frequently associated with renal disease - 12% of patients : proteinuria - 59% of these : nephrotic syndrome Mambranous glomerulonephritis (m/c) Mesangioproliferative glomerulonephritis Minimal change nephrotic syndrome Focal segmental glomerulosclerosis IgM nephropathy, IgA nephropathy Kimura’s disease and NS

Lesions of Kimura’s disease often precede or coincidence with the development of renal involvement In some case, NS and eosinophilia predated diagnosis of kimura’s disease

Relationship between Kimura’s disease and NS Pathogenesis of KD and associated renal involvement - not well understood Various cytokines released from activated T cell -> may also increase permeability of glomerular basement membrane -> leading to proteinuria -> explaining causative association of these two disorder

Treatment of Kimura’s disease and NS Mainstay of treatment for KD is oral steroid In case complicated by renal involvement, treatment with steroids have been tried, with substantial success Cytotoxic agents, cyclosporin, irradiation, surgical removal -> limited success Higher recurrence rate (25%)

대한병리학회지 1995 년 29 권 1 호 기무라병은 주로 동양인에 잘 생기는 만성 염증성 병변으로, 현재 원인이 잘 알려져 있지 않으나, 임상 및 병리학적으로 고유한 특징을 나타내는 질환이다. 한편 이들 환자에서 신 질환이 잘 동반된다고 알려지고 있다. 이에 저자는 신 증후군의 과거력이 있는 환자에서 발생한 기무라병 림프선염 1 예를 경험하고, 이 질환에 대한 이해를 돕고자 이를 보고하고자 한다.

대한내과학회지 1998 년 54 권 5 호 We report a case of IgA nephropathy associated with Kimura’s disease with a review of the literature. 대한소아신장학지 1999 년 3 권 2 호 We report two cases of nephrotic syndrome associated with Kimura disease, and this is the first report of Kimura disease with renal involvement in Korean children.