Pub Date : 2019-05-06DOI: 10.1177/2059300719844991
R. Hanna, Marina Barsoum, A. Vandross, J. Zuckerman, B. Cone, Shih-fan Sun, Anthony Ghobran, Samuel Olanrewaju, Farid Arman, I. Kurtz, U. Selamet
Monoclonal gammopathy is a premalignant condition associated with an abnormal circulating immunoglobulin indicative of an expanded B cell clone. Apart from monitoring, no other intensive management is prescribed in these cases. Periodic bone marrow biopsies when free light chain imbalances are detected are used to spot incidental cases of multiple myeloma. New reports have suggested the existence of a monoclonal gammopathy of renal significance where a circulating antibody and normal bone marrow biopsy results may be associated with proteinuric renal disease due to monoclonal immunoglobulin deposition. We report a case of a 65-year-old male with an immunoglobulin G kappa monoclonal gammopathy of undetermined significance, chronic kidney disease, proteinuria, and an initial inconclusive renal biopsy. His chronic kidney disease worsened with persistence of 0.5 g of proteinuria. Given the finding of ongoing Bence Jones proteinuria, a repeat renal biopsy was done revealing monoclonal immunoglobulin G kappa deposition disease. Bone marrow biopsy showed 20% plasma cells that could be consistent with smoldering myeloma. The patient’s renal disease has stabilized after starting treatment for the monoclonal gammopathy of renal significance. This case illustrates the importance of renal biopsy in making the diagnosis of monoclonal immune deposition disease and monoclonal gammopathy of renal significance.
{"title":"Light and heavy chain deposition revealed by repeat renal biopsy after inconclusive initial biopsy","authors":"R. Hanna, Marina Barsoum, A. Vandross, J. Zuckerman, B. Cone, Shih-fan Sun, Anthony Ghobran, Samuel Olanrewaju, Farid Arman, I. Kurtz, U. Selamet","doi":"10.1177/2059300719844991","DOIUrl":"https://doi.org/10.1177/2059300719844991","url":null,"abstract":"Monoclonal gammopathy is a premalignant condition associated with an abnormal circulating immunoglobulin indicative of an expanded B cell clone. Apart from monitoring, no other intensive management is prescribed in these cases. Periodic bone marrow biopsies when free light chain imbalances are detected are used to spot incidental cases of multiple myeloma. New reports have suggested the existence of a monoclonal gammopathy of renal significance where a circulating antibody and normal bone marrow biopsy results may be associated with proteinuric renal disease due to monoclonal immunoglobulin deposition. We report a case of a 65-year-old male with an immunoglobulin G kappa monoclonal gammopathy of undetermined significance, chronic kidney disease, proteinuria, and an initial inconclusive renal biopsy. His chronic kidney disease worsened with persistence of 0.5 g of proteinuria. Given the finding of ongoing Bence Jones proteinuria, a repeat renal biopsy was done revealing monoclonal immunoglobulin G kappa deposition disease. Bone marrow biopsy showed 20% plasma cells that could be consistent with smoldering myeloma. The patient’s renal disease has stabilized after starting treatment for the monoclonal gammopathy of renal significance. This case illustrates the importance of renal biopsy in making the diagnosis of monoclonal immune deposition disease and monoclonal gammopathy of renal significance.","PeriodicalId":40272,"journal":{"name":"Nephrology @ Point of Care","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/2059300719844991","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"65509546","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-04-02DOI: 10.1177/2059300719834928
S. Naderi, K. Amann, U. Janssen
Renal failure in sarcoidosis is rare and data on its long-term outcome are scarce. To investigate the pattern of renal involvement in sarcoidosis, its clinical course and response to treatment in the long-term. A single-center retrospective study with review of renal biopsies and medical charts was performed. Between January 2005 and December 2016, seven patients with sarcoidosis underwent a kidney biopsy. This is equivalent to a frequency of 1.6% in a total of 434 biopsies from native kidney performed in our institution. All patients presented with renal failure. Five patients had granulomatous interstitial nephritis (GIN) and one patient each interstitial nephritis without granuloma and nephrocalcinosis. Three patients had concomitant glomerular disease: IgA nephropathy (n = 2), membranous and focal proliferative glomerulonephritis (n = 1). Most patients (n = 5) presented with hypercalcemia. All patients initially received oral prednisolone 1 mg/kg/day (n = 3) or 0.5 mg/kg/day (n = 4), respectively, with subsequent tapering or suspension. One patient was started on azathioprine after 18 months to spare steroids. After a mean follow-up of 59 months mean estimated glomerular filtration rate (eGFR) had improved from 19 ± 7 at presentation to 49 ± 16 mL/min. No patient required dialysis. All patients started on prednisolone 1 mg/kg/day developed transient diabetes mellitus while patients on 0.5 mg/kg/day did not. Renal function improvement did not differ between both treatment groups. GIN was the most common diagnosis in sarcoidosis patients with renal failure. Initial hypercalcemia was observed in the majority. Early steroid treatment lead to sustained renal function improvement.
{"title":"Renal involvement in sarcoidosis: Histologic findings and clinical course","authors":"S. Naderi, K. Amann, U. Janssen","doi":"10.1177/2059300719834928","DOIUrl":"https://doi.org/10.1177/2059300719834928","url":null,"abstract":"Renal failure in sarcoidosis is rare and data on its long-term outcome are scarce. To investigate the pattern of renal involvement in sarcoidosis, its clinical course and response to treatment in the long-term. A single-center retrospective study with review of renal biopsies and medical charts was performed. Between January 2005 and December 2016, seven patients with sarcoidosis underwent a kidney biopsy. This is equivalent to a frequency of 1.6% in a total of 434 biopsies from native kidney performed in our institution. All patients presented with renal failure. Five patients had granulomatous interstitial nephritis (GIN) and one patient each interstitial nephritis without granuloma and nephrocalcinosis. Three patients had concomitant glomerular disease: IgA nephropathy (n = 2), membranous and focal proliferative glomerulonephritis (n = 1). Most patients (n = 5) presented with hypercalcemia. All patients initially received oral prednisolone 1 mg/kg/day (n = 3) or 0.5 mg/kg/day (n = 4), respectively, with subsequent tapering or suspension. One patient was started on azathioprine after 18 months to spare steroids. After a mean follow-up of 59 months mean estimated glomerular filtration rate (eGFR) had improved from 19 ± 7 at presentation to 49 ± 16 mL/min. No patient required dialysis. All patients started on prednisolone 1 mg/kg/day developed transient diabetes mellitus while patients on 0.5 mg/kg/day did not. Renal function improvement did not differ between both treatment groups. GIN was the most common diagnosis in sarcoidosis patients with renal failure. Initial hypercalcemia was observed in the majority. Early steroid treatment lead to sustained renal function improvement.","PeriodicalId":40272,"journal":{"name":"Nephrology @ Point of Care","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/2059300719834928","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41576677","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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