Diagnosis and treatment of a patient with Kimura’s disease associated with nephrotic syndrome and lymphadenopathy of the epitrochlear nodes

BMC Nephrology, Feb 2015

Background Kimura's disease (KD) is a slowly progressing rare, benign inflammatory disorder of the soft tissues. It typically presents as subcutaneous tumor-like nodules, located most frequently in the head and neck region. KD is often accompanied by increased peripheral eosinophilia and elevated levels of serum immunoglobulin (Ig) E. There is renal involvement in approximately 12-16% of KD cases. We report the case of a 23-year-old Chinese man who was found to have KD associated with nephrotic syndrome. Case presentation A 23-year-old Chinese man presented with edema in both legs and a mass in ulnar side of his right upper arm on August 8th 2013. Before admission to our hospital, an ultrasound examination revealed swollen lymph nodes in the medial aspect of his right upper arm, proximal to the elbow. The patient was admitted on August 19th 2013 as a result of edema, severe proteinuria, and low serum albumin levels. He had a white blood cell count of 7.7 × 109 cells/L, 48.5% eosinophils, 4+ albuminuria, 24-hour urinary protein excretion 9.3 g, serum protein 50.3 g/L; serum albumin 16 g/L and IgE 1,510 IU/ml. A biopsy of the epitrochlear nodes revealed eosinophilic hyperplastic lymphogranulomatous tissue. A renal biopsy indicated focal segmental glomerulosclerosis (FSGS) (cellular variant) with no infiltration of eosinophil in renal interstitium. The results of immune-staining on the renal biopsy were negative for IgG, IgA, IgM, C3 and C1q. The electron microscopic analysis showed podocyte effacement. His final diagnosis was Kimura's disease associated with nephrotic syndrome. He received methylprednisolone therapy as well as symptomatic treatment, and was discharged with key indicators in normal range on September 17th 2013. During the year following, he had methylprednisolone at a maintenance dose of 8 mg/day, and no relapses occurred up to now. Conclusion Methylprednisolone therapy is effective in KD associated with nephrotic syndrome, and long-term administration of methylprednisolone at maintenance dose may be a way to prevent relapses of KD.

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Diagnosis and treatment of a patient with Kimura’s disease associated with nephrotic syndrome and lymphadenopathy of the epitrochlear nodes

Zhu et al. BMC Nephrology Diagnosis and treatment of a patient with Kimura's disease associated with nephrotic syndrome and lymphadenopathy of the epitrochlear nodes Sheng-lang Zhu 0 Peng-fei Wei 1 Jie-hui Chen 0 Zhen-fu Zhao 1 Qian-na Xu 0 Ling Ye 0 0 Department of Nephrology, Nanshan Hospital, Shenzhen University , 89 Taoyuan Road, Shenzhen 518052 , P. R. China 1 Health Science Center, Shenzhen University , Shenzhen , China Background: Kimura's disease (KD) is a slowly progressing rare, benign inflammatory disorder of the soft tissues. It typically presents as subcutaneous tumor-like nodules, located most frequently in the head and neck region. KD is often accompanied by increased peripheral eosinophilia and elevated levels of serum immunoglobulin (Ig) E. There is renal involvement in approximately 12-16% of KD cases. We report the case of a 23-year-old Chinese man who was found to have KD associated with nephrotic syndrome. Case presentation: A 23-year-old Chinese man presented with edema in both legs and a mass in ulnar side of his right upper arm on August 8th 2013. Before admission to our hospital, an ultrasound examination revealed swollen lymph nodes in the medial aspect of his right upper arm, proximal to the elbow. The patient was admitted on August 19th 2013 as a result of edema, severe proteinuria, and low serum albumin levels. He had a white blood cell count of 7.7 109 cells/L, 48.5% eosinophils, 4+ albuminuria, 24-hour urinary protein excretion 9.3 g, serum protein 50.3 g/L; serum albumin 16 g/L and IgE 1,510 IU/ml. A biopsy of the epitrochlear nodes revealed eosinophilic hyperplastic lymphogranulomatous tissue. A renal biopsy indicated focal segmental glomerulosclerosis (FSGS) (cellular variant) with no infiltration of eosinophil in renal interstitium. The results of immune-staining on the renal biopsy were negative for IgG, IgA, IgM, C3 and C1q. The electron microscopic analysis showed podocyte effacement. His final diagnosis was Kimura's disease associated with nephrotic syndrome. He received methylprednisolone therapy as well as symptomatic treatment, and was discharged with key indicators in normal range on September 17th 2013. During the year following, he had methylprednisolone at a maintenance dose of 8 mg/day, and no relapses occurred up to now. Conclusion: Methylprednisolone therapy is effective in KD associated with nephrotic syndrome, and long-term administration of methylprednisolone at maintenance dose may be a way to prevent relapses of KD. Kimura's disease; Nephrotic syndrome; Eosinophilia; Immunoglobulin E; Glucocorticoid - Background Kimuras disease (KD) is a rare, chronic inflammatory disorder that affects the lymph nodes, soft tissues, and salivary glands. It occurs predominantly in Asian males with a male to female ratio of approximately 9.1:3.5 [1]. There are about 300 reported cases globally, with most occurring in Asia [2]. The etiology of KD has yet to be resolved. The characteristic manifestations of KD include subcutaneous tumor-like masses in the head and neck region, as well as subcutaneous nodules with eosinophilic lymphoid granulomas. Multiple organs can be affected, with about 12-16% of patients suffering renal involvement [3]. Indeed, when there is renal involvement, the disease may present initially as a nephrotic syndrome. KD is diagnosed after the exclusion of malignancies (e.g., T-cell lymphoma and Hodgkins disease [1,3]) based on the presence of a painless subcutaneous tumor-like mass, increased immunoglobulin (Ig)E levels, eosinophilia and a biopsy that shows the abnormal proliferation of lymphoid follicles and vascular endothelium. Here, we present the case of a patient with KD associated with nephrotic syndrome characterized by focal segmental glomerulosclerosis (FSGS). We also provide an analysis of the diagnostic strategy for recognizing KD associated with nephrotic syndrome, as well as treatments and methods to prevent the relapse of this condition. Case presentation A 23-year-old Chinese man presented with edema in both legs and a mass in ulnar side of his right upper arm on August 8th 2013. The patient reported that the edema had been present for 11 days and that the mass had been palpable for 3 years, and was first detected in October 2010. According to the patients recollection, it had apparently increased in size since June 2013 with accompanying warmth of the overlying skin. The patient had not received any prior treatment for these conditions. Before admission to our hospital, the patient developed worsened lower limb edema, abdominal distention, and oliguria. Two days later, an ultrasound examination revealed swollen lymph nodes in the medial aspect of his right upper arm, proximal to the elbow. Because the appearance of the swollen nodes was consistent with inflammation, the patient was prescribed cefprozil, which resulted in a reduction in the swelling. The patient was admitted on August 19th 2013 as a result of edema, severe (...truncated)


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Sheng-lang Zhu, Peng-fei Wei, Jie-hui Chen, Zhen-fu Zhao, Qian-na Xu, Ling Ye. Diagnosis and treatment of a patient with Kimura’s disease associated with nephrotic syndrome and lymphadenopathy of the epitrochlear nodes, BMC Nephrology, 2015, pp. 10, 16, DOI: 10.1186/s12882-015-0007-7