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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2015  |  Volume : 26  |  Issue : 1  |  Page : 115-118
Mesangioproliferative glomerulonephritis in a patient with Kimura's disease presenting as Nephrotic syndrome

1 Department of Nephrology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India
2 Department of Medicine, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India
3 Department of Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India

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Date of Web Publication8-Jan-2015


Kimura's disease is a rare chronic eosinophilic inflammatory disorder of unknown etiology. Majority of cases have been reported from South East Asia, while sporadic occurrences have been reported worldwide, including the Indian subcontinent. Nephrotic syndrome may be the presenting manifestation of Kimura's disease, and a variety of renal lesions are observed histologically in such patients. We herein describe a case of steroid-responsive mesangioproliferative glomerulonephritis related to kimura's disease.

How to cite this article:
Rathore SS, Arora P, Usha, Prakash J. Mesangioproliferative glomerulonephritis in a patient with Kimura's disease presenting as Nephrotic syndrome. Saudi J Kidney Dis Transpl 2015;26:115-8

How to cite this URL:
Rathore SS, Arora P, Usha, Prakash J. Mesangioproliferative glomerulonephritis in a patient with Kimura's disease presenting as Nephrotic syndrome. Saudi J Kidney Dis Transpl [serial online] 2015 [cited 2021 Dec 3];26:115-8. Available from: https://www.sjkdt.org/text.asp?2015/26/1/115/148756

   Introduction Top

The first case of Kimura's disease was reported by Kimm and Szeto from China in 1937; however, it got widespread recognition as a specific disease entity only after 1948 when Kimura et al reported similar cases from Japan. [1] Kimura's disease usually presents with a subcutaneous swelling and associated lymphadenopathy in the periauricular area. Kimura's disease has a predilection to affect the kidneys. Proteinuria has been reported in 12-16% cases, and approximately 60-70% of them have nephrotic range proteinuria. [2] Nephrotic syndrome in relation to Kimura's disease has been reported in 41 cases so far. [3] We are reporting a case of steroid-responsive mesangioproliferative glomerulonephritis (MsPGN) in a patient with kimura's disease without recurrence of nephrotic syndrome over a follow-up period of five years.

   Case Report Top

An 18-year-old male patient presented to us in 2006 with history of anasarca for the last one year, which used to be partially relieved with oral diuretics. It was associated with recurrent, self-limiting gross hematuria episodes. On examination, he had gross edema of both lower limbs. He was normotensive and the rest of the general physical and systemic examination was normal except for the presence of a 4 cm size scar mark behind the right ear. When probed further; he revealed history of presence of a swelling in that area for the last 10 years. The swelling was of around plum size, non-progressive, non-pruritic with normal overlying skin. The lesion had been excised 10 months ago (2 months after surgery, the patient first noticed edema of both feet) and histological examination of excised lymph node showed preservation of lymph node architecture with hyperplastic follicles in the superficial cortex. The para-cortex showed vascular proliferation with intense eosinophilic infiltration. Some of the sections showed formation of eosinophilic abscess. Histology findings were consistent with diagnosis of Kimura's disease.

Laboratory investigations revealed hemoglobin of 13.8 g/dL with total leukocyte count of 13,500/μL and differential leukocyte count of 80% neutrophils, 16% lymphocytes and 3% eosinophils. Blood urea and serum creatinine levels were 27 mg/dL and 0.7 mg/dL, respectively. Serum electrolytes were normal. Urine analysis showed 4 plus proteinuria, nil sugar, nil cells and presence of hyaline casts. Serum albumin was low (2.1 g/dL), with normal levels of SGOT, SGPT, alkaline phosphatase and total bilirubin. Serum cholesterol and triglyceride levels were high, with values of 440 mg/dL and 387 mg/dL, respectively. The 24-h urine examination revealed proteinuria of 8.6 g/day. Assays for hepatitis B surface antigen, hepatitis C virus and HIV were negative. Complement C3 level was low; 16.3 mg/dL (normal range: 20-40 mg/dL), while the C4 level was normal; 115.3 mg/dL (normal range: 80-160 mg/dL). Anti nuclear antibody assay was negative. Ultrasound examination showed normal-sized kidneys with no evidence of urolithiasis or structural abnormality of the urinary tract.

The patient underwent renal biopsy and the specimen contained eight glomeruli. On light microscopy, some of the glomeruli were normal while majority of them showed mild glomerulomegaly, increased urinary space and mesangial proliferation. In some glomeruli, endocapillary proliferation was seen. Cellular crescent was seen in one glomerulus. Few tubules were packed with inflammatory cell casts. Infiltration with mono-nuclear cells was noted in the interstitium. Some blood vessels showed inflammatory cell infiltration along with smooth muscle proliferation. The findings were suggestive of diffuse proliferative glomerulonephritis, predominantly of the mesangioproliferative type along with small vessel vasculitis. Prednisolone at a dose of 1 mg/kg/day was started, along with oral diuretics. After eight weeks of treatment, proteinuria decreased dramatically to 409 mg/day. At this point of time, it was decided to taper off the steroid dose and steroids were completely withdrawn gradually over a span of the next six weeks. The patient remained in our follow-up for five years, during which there was no clinical or laboratory evidence of relapse of nephrotic syndrome. Similarly, there was no recurrence of skin lesions or lymphadenopathy. The present case of Kimura's disease had certain unique features such as (1) development of nephrotic syndrome occurring very late, 10 years after onset of lymphadenopathy, (2) sustained remission of nephrotic syndrome with oral prednisolone therapy and (3) there was no recurrence of either nephrotic syndrome or kimura's disease during the follow-up period of five years.

   Discussion Top

Kimura's disease classically affects young males of oriental region and presents with subcutaneous swelling around the head and neck area, especially periauricular one. Axillary, epitrochlear and inguinal lymph nodes and submandibular salivary glands are other commonly affected sites. Rarely, it may involve nasal sinuses, oral cavity, orbit, median nerve and lacrimal glands. [4] The lesions are classically single or multiple, painless, often pruritic and, many times, recurrent. In majority of the cases, a definitive diagnosis can only be made after excision biopsy. Histologically, lymphoid hyperplasia with abundant germinal center proliferation along with post-capillary venule proliferation in the paracortex and variable amount of fibrosis are typically seen. Sometimes, Warthin-Finkeldey type of polykaryocytes are found. [5],[6] However, the most characteristic finding is marked eosinophilic infiltration of the germinal centers (folliculolysis), sometimes with microabscess formation as seen in the present case. Other important laboratory findings are presence of peripheral eosinophilia and increased serum immunoglobulin E levels, which are seen in more than 90% of the cases. [7] Correlation between degree of eosinophilia and size of soft tissue swellings has been suggested. Eosinophilia was not seen in the present case, which can be explained on this basis as skin lesions had been excised a long time ago.

Pathogenesis of Kimura's disease remains elusive but there is strong evidence of altered Tcell immunoregulation playing a central role. [8] Nephrotic syndrome in patients with kimura's disease is mostly reported from oriental region. However, occurrence of nephrotic syndrome in patients with Kimura's disease is rarely reported outside the oriental region. To the best of our knowledge, this is only the second case being reported from the Indian subcontinent; the first case of nephrotic syndrome in relation to Kimura's disease was reported from Chandigarh in 1996 and kidney histology in this report showed features of minimal change disease. [9] A variety of histological lesions including minimal change disease, MsPGN, focal segmental glomerulonephritis, IgM nephropathy, IgA nephropathy and membranous nephropathy, have been described in association with Kimura's disease. [3] In the literature, membranous glomerulonephritis has been described as the most common histological lesion associated with kimura's disease. [10] However, a recent study of 20 cases of Kimura's disease-associated nephrotic syndrome in the Chinese literature (1984-2007) noted that MsPGN was the most common histological lesion on renal biopsy, seen in 9/13 (69%) specimens. [11] The skin lesions classically precede the appearance of nephropathy as seen in the present case; however, it may develop concurrently or months later, but they usually have a close temporal relation, suggesting a common pathogenic link between Kimura's disease and the nephropathy.

An optimal treatment protocol for nephropathy is not well defined, largely due to the rarity of lesions and lack of prospective follow-up. Nephrotic syndrome in patients with Kimura's disease is usually treated with oral prednisolone. Response to treatment is variable, depending on renal histology. Remission of proteinuria was best described in patients with minimal change disease and MsPGN. [3],[11] Our patient responded well to steroids and there was no recurrence of either skin lesions or nephrotic syndrome during the follow-up period of five years.

To the best of our knowledge, we are reporting the second case of nephrotic syndrome associated with Kimura's disease from India. Renal histology revealed MsPGN and nephrotic syndrome achieved complete remission with oral steroid therapy without recurrence over a followup period of five years. Kimura's disease should be suspected in a patient of nephrotic syndrome who presents with or has a history of subcutaneous swelling around the peri auricular area.

Conflict of interest: None

   References Top

Kimura T, Yoshimura S, Ishikawa E. On the unusual granulation combined with hyperplastic changes of lymphatic tissues. Trans Soc Pathol Jpn 1948;37:179-80.  Back to cited text no. 1
Yamada A, Mitsuhashi K, Miyakawa Y, et al. Membranous glomerulonephritis associated with eosinophilic lymphfolliculosis of the skin (Kimura's disease): Report of a case and review of the literature. Clin Nephrol 1982;18:211-5.  Back to cited text no. 2
Yuen SK, Yong SP, Tsui WM, Siu FY, Tsui HS, Cheung KO. Minimal change Nephrotic Syndrome with IgM deposits in Kimura's Disease: A case report and literature review. Hong Kong J Nephrol 2004;6:97-102.  Back to cited text no. 3
Sun QF, Xu DZ, Pan SH, et al. Kimura disease: Review of literature. Intern Med J 2008;38:668-74.  Back to cited text no. 4
Kung ITM, Gibson JB, Bannatyne PM. Kimura disease: A clinicopathological study of 21 cases and its distinction from angiolymphoid hyperplasia with eosinophilia. Pathology 1984;16:39-44.  Back to cited text no. 5
Motoi M, Wahid S, Horie Y, Akagi T. Kimura's disease: Clinical, histological, and immunological studies. Acta Med Okayama 1992;46:449-55.  Back to cited text no. 6
Armstrong WB, Alison G, Pena F, Kim JK. Kimura's disease two case reports and literature review. Ann Otol Rhinol Laryningol 1998;107:1066-71.  Back to cited text no. 7
Rajpoot DK, Pahl M, Clark J. Nephrotic syndrome associated with kimura disease. Pediatr Nephrol 2000;14:486-8.  Back to cited text no. 8
Sud K, Saha T, Das A, et al. Kimura's disease and minimal change nephrotic syndrome. Nephrol Dial Transplant 1996;11:1349-51.  Back to cited text no. 9
Natov SN, Strom JA, Ucci A. Relapsing nephrotic syndrome in a patient with Kimura's disease and IgA glomerulonephritis. Nephrol Dial Transplant 1998;13:2358-63.  Back to cited text no. 10
DY, Mao JH, Zhang Y, et al. Kimura disease: A case report and review of the Chinese literature. Nephron Clin Pract 2009;111:c55-61.  Back to cited text no. 11

Correspondence Address:
Dr. Surendra Singh Rathore
Department of Nephrology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh
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DOI: 10.4103/1319-2442.148756

PMID: 25579728

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