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      Kimura’s disease with membranoproliferative glomerulonephritis: a case report with literature review

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          Abstract

          Background: Kimura's disease is a rare disease and its etiology is still unclear. Here we reported a case with lymphadenopathy complicated with secondary membranoproliferative glomerulonephritis.

          Case presentation: A 46-year-old Chinese man presented with bilateral tumor-like nodules over his neck during the past 6 months and developed edema for 15 days. His blood pressure was 145/90 mmHg, multiple 1 × 1 cm masses were found over bilateral post-auricular and submandibular areas, along with trace edema of the lower extremities. Laboratory data showed an increased peripheral eosinophil count at 3.66 × 10 9/L (50% of total leukocytes), with a 24-hour urine total protein of 8 g and a serum albumin of 19 g/L, and serum IgE of 2930 IU/ml (<100 IU/ml). The patient underwent renal biopsy, which revealed membranoproliferative glomerulonephritis with eosinophilic infiltration of the interstitium. Lymph node biopsy showed eosinophilic lymphoid follicular granuloma. Bone marrow biopsy showed no abnormalities. A diagnosis of Kimura's disease was then established. We started him on prednisone 60 mg/day (1 mg/kg), and tapered the dose to 55 mg/day 2 months later, followed by a gradual reduction of 2.5 mg every 2 weeks. Valsartan was given for blood pressure control. His neck nodules shrank after 2 weeks of treatment and complete renal remission was achieved 3 months later. No relapse occurred after follow-up for 31 months.

          Conclusion: Kimura's disease can present with bilateral neck nodules and nephrotic syndrome (membranoproliferative glomerulonephritis), and prednisone can be a suitable choice of treatment.

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          Most cited references 15

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          Idiopathic membranoproliferative glomerulonephritis: does it exist?

          When membranoproliferative glomerulonephritis (MPGN) was first delineated as a discrete clinico-pathological entity more than a half-century ago, most cases were regarded as idiopathic (or primary) in nature. Advances in analysis of pathogenetic mechanisms and etiologies underlying the lesion of MPGN have radically altered the prevalence of the truly idiopathic form of MPGN. In addition, MPGN as a category among renal biopsies showing glomerulonephritis has diminished over time. In the modern era, MPGN is mainly classified morphologically on the basis of immunoglobulin (Ig; monoclonal or polyclonal) and complement (C3 only or combined with Ig) deposition and secondarily on the basis of its appearance on ultra-structural examination. Idiopathic MPGN is a diagnosis of exclusion, at least in many adults and a portion of children, and a systematic approach to evaluation will often uncover a secondary cause, such as an infection, autoimmune disease, monoclonal gammopathy, neoplasia, complement dysregulation or a chronic thrombotic microangiopathy. Idiopathic MPGN remains an 'endangered species' after its separation from these known causes.
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            Increase of Th2 and Tc1 cells in patients with Kimura's disease.

            A Th1/Th2 cytokine imbalance with a predominance of Th2 cytokines has been suggested to be of pathogenic importance in Kimura's disease.
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              Membranous glomerulonephritis associated with eosinophilic lymphfolliculosis of the skin (Kimura's disease): report of a case and review of the literature.

              A 48-year-old male, who had been suffering from eosinophilic lymphfolliculosis of the skin (Kimura's disease) for six years, developed the nephrotic syndrome. Renal biopsy revealed immunopathological and electron microscopic changes characteristic of membranous glomerulonephritis. A review of the literature disclosed a high frequency of renal involvement in Kimura's disease. Out of 175 reported cases of the disease, 21 (12%) were found to have proteinuria including 13 who had the nephrotic syndrome. Some of the patients were noticed to have proteinuria before the onset of the skin lesions, suggesting the existence of common etiological factor(s) capable of inducing both skin and kidney lesions.
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                Author and article information

                Journal
                Ren Fail
                Ren Fail
                IRNF
                irnf20
                Renal Failure
                Taylor & Francis
                0886-022X
                1525-6049
                2019
                26 March 2019
                : 41
                : 1
                : 126-130
                Affiliations
                [a ]Department of Nephrology, the First Hospital of Jilin University , Changchun, China;
                [b ]Department of Digestive Endoscopy, the Second Hospital of Jilin University , Changchun, China
                Author notes
                CONTACT Li Zhang zhanglinephrology@ 123456163.com Department of Nephrology, the First Hospital of Jilin University , 71 Xinmin Street, Changchun, China
                Article
                1584115
                10.1080/0886022X.2019.1584115
                6442089
                30912457
                © 2019 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                Page count
                Figures: 4, Tables: 0, Pages: 6, Words: 2262
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                Clinical Study

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