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10.1186/s12882-017-0561-2

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suck abstract from ncbi


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pmid28446143
      BMC+Nephrol 2017 ; 18 (1 ): 139
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  • IgG4-related membranous glomerulonephritis and generalized lymphadenopathy without pancreatitis: a case report #MMPMID28446143
  • Huart J ; Grosch S ; Bovy C ; Moutschen M ; Krzesinski JM
  • BMC Nephrol 2017[Apr]; 18 (1 ): 139 PMID28446143 show ga
  • BACKGROUND: IgG4-related disease is a recently described pathologic entity. This is the case of a patient with nephrotic syndrome and lymphadenopathy due to IgG4-related disease. Such a kidney involvement is quite peculiar and has only been described a few times recently. Renal biopsy showed a glomerular involvement with membranous glomerulonephritis in association with a tubulo-interstitial nephropathy. Moreover, the patient was not suffering from pancreatitis. CASE PRESENTATION: The patient is a middle-aged man of Moroccan origin. He has developed recurrent episodes of diffuse lymphadenopathies, renal failure and nephrotic syndrome. Renal biopsies showed membranous glomerulonephritis. DISCUSSION AND CONCLUSION: The diagnostic approach of this atypical presentation is discussed in this case report as well as diagnostic criteria, therapeutic strategies, biomarkers and pathophysiology of IgG4-related disease. IgG4-related membranous glomerulonephritis is a well-established cause of membranous glomerulonephritis. It must be sought after in every patient with a previous diagnosis of IgG4-related disease and in every patient with this histological finding on renal biopsy. Corticoids are still the first-line treatment of IgG4-related disease. New therapeutic strategies are needed to avoid glucocorticoids long term side-effects. Interestingly, the patient was prescribed cyclophosphamide in addition to glucocorticoids for an immune thrombocytopenia. This treatment had a very good impact on his IgG4-related disease.
  • |Adult [MESH]
  • |Diagnosis, Differential [MESH]
  • |Glomerulonephritis, Membranous/*diagnosis/*drug therapy/immunology [MESH]
  • |Glucocorticoids/*therapeutic use [MESH]
  • |Humans [MESH]
  • |Immunoglobulin G/*immunology [MESH]
  • |Lymphadenopathy/*diagnosis/*drug therapy/immunology [MESH]
  • |Male [MESH]
  • |Pancreatitis/diagnosis/drug therapy/immunology [MESH]


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