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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
CASE REPORT  
Year : 2019  |  Volume : 30  |  Issue : 1  |  Page : 254-257
Minimal change disease and Kimura's disease responding to tacrolimus therapy


1 Department of Nephrology, Jawaharlal Nehru Medical College, Wardha, Maharashtra, India
2 Department of Nephrology, Government Medical College and Super Speciality Hospital, Nagpur, Maharashtra, India
3 Department of Medicine, Northern Railway Central Hospital, New Delhi, India

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Date of Submission13-Jan-2018
Date of Decision18-Feb-2018
Date of Acceptance20-Feb-2018
Date of Web Publication26-Feb-2019
 

   Abstract 


Kimura's disease (KD) usually presents with a subcutaneous swelling and associated lymphadenopathy in the periauricular area. KD has a tendency to involve the kidneys. Proteinuria is reported in 12%–16% of cases, and around 60%–70% of them develop nephrotic range proteinuria. We are reporting a case of KD which developed around 12 years later in a patient of biopsy-proven steroid responsive minimal change disease. Recurrent swellings of KD and subnephrotic range proteinuria responded to low-dose tacrolimus therapy (0.05 mg/kg).

How to cite this article:
Balwani MR, Bawankule CP, Pasari A, Tolani P, Vakil S, Yadav R. Minimal change disease and Kimura's disease responding to tacrolimus therapy. Saudi J Kidney Dis Transpl 2019;30:254-7

How to cite this URL:
Balwani MR, Bawankule CP, Pasari A, Tolani P, Vakil S, Yadav R. Minimal change disease and Kimura's disease responding to tacrolimus therapy. Saudi J Kidney Dis Transpl [serial online] 2019 [cited 2019 Jul 20];30:254-7. Available from: http://www.sjkdt.org/text.asp?2019/30/1/254/252921



   Introduction Top


Kimura's disease (KD) was reported by Kimm and Szeto from China in 1937; but, it was widely recognized as a specific disease entity only when Kimura et al reported similar cases from Japan after 1948.[1] KD usually presents with a subcutaneous swelling and associated lymphadenopathy in the periauricular area. KD tends to involve the kidneys. Proteinuria is reported in 12%–16% of cases, and around 60%–70% of them develop nephrotic range proteinuria.[2] We are reporting a case of Kimura disease which developed around 12 years later in a patient of biopsy-proven steroid responsive minimal change disease. Recurrent swellings of KD responded to low-dose tacrolimus therapy (0.05 mg/kg).


   Case Report Top


An 18-year-old male patient was admitted in year 2002 with a history of anasarca for the last four months. 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. Renal biopsy was performed in view of nephrotic range proteinuria and serum hypoalbuminemia which showed minimal change disease. He responded to six months oral steroid therapy and was off steroids afterward with nil albuminuria. In February 2014, he had a 3 cm × 4 cm cystic swelling behind the right ear which was freely mobile, pruritic, palpable, and nontender. Laboratory investigations revealed hemoglobin of 14.8 g/dL with total leukocyte count of 4500/μL and differential leukocyte count of 62% neutrophils, 12% eosinophils, 24% lymphocytes and 2% monocytes. Serum IgE levels were elevated. Blood urea and serum creatinine levels were 16 mg/dL and 0.6 mg/dL, respectively. Serum electrolytes were normal. Urine analysis showed +3 albumin, nil sugar and nil cells/hpf. 24-h urine protein was 3.6 g/day. Serum albumin was 2.1 g/dL, with normal levels of aspartate transaminase (AST), alanine transaminase (ALT), alkaline phosphatase, and total bilirubin. Serum cholesterol level was 440 mg/dL with other lipid profile parameters normal. Assays for hepatitis B surface antigen, hepatitis C virus, and HIV were negative. Complement C3 and C4 levels were normal. Ultrasound examination showed normal-sized kidneys with of maintained corticomedullary differentiation. The patient was not willing to undergo renal biopsy. Histopathology of the surgically excised tumor revealed preservation of lymph node architecture with hyperplastic follicles in the superficial cortex. The paracortex showed vascular proliferation with intense eosinophilic infiltration. Few sections showed the formation of eosinophilic abscess [Figure 1]. Histology findings were consistent with diagnosis of KD. A variable number of mononuclear cells and lymphoid follicle formation was also seen. The patient was diagnosed with KD with nephrotic syndrome. Nephrotic syndrome responded to 6 months empirical oral steroid therapy with nil albuminuria till May 2015. In March 2015, he again developed similar swelling in the right face which was submucosal, pruritic with normal overlying skin. Again the excised tumor histopathology showed KD. In May 2015, he developed another swelling of 4 cm × 3 cm size behind left ear, which was progressive in size, pruritic, and non tender. Laboratory investigations revealed hemoglobin of 14.4 g/dL with total leukocyte count of 6500/μL and a differential leukocyte count of 58% neutrophils, 14% eosinophils, 26% lymphocytes, and 2% monocytes. Serum Ig E levels were elevated. Blood urea and serum creatinine levels were 18 mg/dL and 0.8 mg/dL, respectively. Serum electrolytes were normal. Urine analysis showed +2 albumin, nil sugar, and nil cells/hpf. 24-h urine protein was 0.8 g/day. Serum albumin was 3.8 g/dL, with normal levels of AST, ALT, alkaline phosphatase, and total bilirubin. Serum cholesterol level was 210 mg/dL with other lipid profile parameters normal. The patient was started on 0.05 mg/kg/day tacrolimus without steroids for six months and swelling decreased in size from 4 cm × 3 cm to 1 cm × 1 cm within a month of starting treatment. Our target tacrolimus trough level was 4–6 ng/mL. No significant tacrolimus therapy-related side effects were seen. No new swellings were observed during this six months posttreatment follow-up.
Figure 1: Eosinophilic abscess with plasma cell and lymphocytes (yellow arrow).

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   Discussion Top


KD usually affects oriental region young males and presents with subcutaneous swelling around the head-and-neck area, especially periauricular one. Epi-trochlear, axillary, inguinal lymph nodes, and submandibular salivary glands are other commonly involved sites. Rarely, it may affect oral cavity, orbit, nasal sinuses, median nerve, and lacrimal glands.[3] The lesions are usually single or multiple, pruritic, often painless, and recurrent. In most of cases, a confirmatory diagnosis can only be made after excision biopsy. Histologically, lymphoid hyperplasia with abundant germinal center proliferation along with postcapillary venule proliferation in the paracortex and variable amount of fibrosis is generally seen. Warthin–Finkeldey type of polykaryocytes may be seen.[4],[5] However, the most characteristic finding is marked eosinophilic infiltration of the germinal centers (folliculolysis), sometimes with microabscess formation as seen in the present case. Peripheral eosinophilia and increased serum immunoglobulin E levels are seen in >90% of the cases.[6] Correlation between degree of eosinophilia and size of soft-tissue swellings has been suggested. Pathogenesis of KD remains unestablished, but there is evidence of T-cell immuno-dysregulation playing an pathogenetic role.[7] Nephrotic syndrome in patients with KD is reported from the oriental region. However, occurrence of nephrotic syndrome in patients with KD is rarely reported outside the oriental region. To the best of our knowledge, this is the first case of KD being reported from the Indian subcontinent who has been successfully treated with low dose tacrolimus therapy. A variety of histological lesions, including minimal change disease, focal segmental glome-rulonephritis, IgM nephropathy, MsPGN, IgA nephropathy, and membranous nephropathy, have been described in association with KD.[8] However, a recent study of 20 cases of KD-associated nephritic 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.[9] In the literature, membranous glomerulonephritis has been described as the most common histological lesion associated with KD.[10] The skin lesions classically precede the appearance of nephropathy as contrast to the present case; however, it may develop concurrently or months later, but they usually have a close temporal relation, suggesting a common link of pathogenesis between the two. An optimal therapy for nephropathy or KD is not well-defined, largely due to the rarity of lesions and loss of follow-up. Nephrotic syndrome in patients with KD is mainly treated with oral prednisolone. Treatment response is variable, depending on renal histology. Remission of proteinuria was best described in patients with minimal change disease and MsPGN.[8],[9] The recurrence of disease is affected by various factors such as disease duration time, lesion diameter, blood eosinophil count, well-defined lesion boundaries, serum IgE levels, and single or multiple lesions.[11] Our patient's skin lesions and subnephrotic range proteinuria responded well to low-dose tacrolimus 0.05 mg/kg/day therapy and there was no recurrence of either skin lesions or proteinuria during the follow-up period of six months. KD should be suspected in a patient of nephritic syndrome who presents with or has a history of subcutaneous swelling around the periauricular area. Tacrolimus may be an effective treatment for patients with KD, but more research is needed to determine its long-term efficacy and safety as well as its mechanism of action.

Conflict of interest:

None declared.



 
   References Top

1.
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
    
2.
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
    
3.
Sun QF, Xu DZ, Pan SH, et al. Kimura disease: Review of the literature. Intern Med J 2008;38:668-72.  Back to cited text no. 3
    
4.
Kung IT, Gibson JB, Bannatyne PM. Kimura's disease: A clinico-pathological study of 21 cases and its distinction from angiolymphoid hyperplasia with eosinophilia. Pathology 1984; 16:39-44.  Back to cited text no. 4
    
5.
Motoi M, Wahid S, Horie Y, Akagi T. Kimura's disease: Clinical, histological and immunohistochemical studies. Acta Med Okayama 1992;46:449-55.  Back to cited text no. 5
    
6.
Armstrong WB, Allison G, Pena F, Kim JK. Kimura's disease: Two case reports and a literature review. Ann Otol Rhinol Laryngol 1998;107:1066-71.  Back to cited text no. 6
    
7.
Rajpoot DK, Pahl M, Clark J. Nephrotic syndrome associated with Kimura disease. Pediatr Nephrol 2000;14:486-8.  Back to cited text no. 7
    
8.
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. 8
    
9.
Wang 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. 9
    
10.
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
    
11.
Lin YY, Jung SM, Ko SF, et al. Kimura's disease: Clinical and imaging parameters for the prediction of disease recurrence. Clin Imaging 2012;36:272-8.  Back to cited text no. 11
    

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Correspondence Address:
Manish R Balwani
Department of Nephrology, Jawaharlal Nehru Medical College, Sawangi, Wardha, Maharashtra
India
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DOI: 10.4103/1319-2442.252921

PMID: 30804291

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