Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney Disease
We report the case of a 38-year-old male with autosomal-dominant polycystic kidney disease (ADPKD) and concomitant nephrotic syndrome secondary to membranous nephropathy (MN). A 3-month course of prednisone 60 mg daily and losartan 100 mg daily resulted in resistance. Treatment with chlorambucil 0.2...
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| Format: | Article |
| Language: | English |
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Wiley
2011-01-01
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| Series: | The Scientific World Journal |
| Online Access: | http://dx.doi.org/10.1100/tsw.2011.94 |
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| author | Ramón Peces Jorge Martínez-Ara Carlos Peces Mariluz Picazo Emilio Cuesta-López Cristina Vega Sebastián Azorín Rafael Selgas |
| author_facet | Ramón Peces Jorge Martínez-Ara Carlos Peces Mariluz Picazo Emilio Cuesta-López Cristina Vega Sebastián Azorín Rafael Selgas |
| author_sort | Ramón Peces |
| collection | DOAJ |
| description | We report the case of a 38-year-old male with autosomal-dominant polycystic kidney disease (ADPKD) and concomitant nephrotic syndrome secondary to membranous nephropathy (MN). A 3-month course of prednisone 60 mg daily and losartan 100 mg daily resulted in resistance. Treatment with chlorambucil 0.2 mg/kg daily, low-dose prednisone, plus an angiotensin-converting enzyme inhibitor (ACEI) and an angiotensin II receptor blocker (ARB) for 6 weeks resulted in partial remission of his nephrotic syndrome for a duration of 10 months. After relapse of the nephrotic syndrome, a 13-month course of mycophenolate mofetil (MFM) 2 g daily and low-dose prednisone produced complete remission for 44 months. After a new relapse, a second 24-month course of MFM and low-dose prednisone produced partial to complete remission of proteinuria with preservation of renal function. Thirty-six months after MFM withdrawal, complete remission of nephrotic-range proteinuria was maintained and renal function was preserved. This case supports the idea that renal biopsy is needed for ADPKD patients with nephrotic-range proteinuria in order to exclude coexisting glomerular disease and for appropriate treatment/prevention of renal function deterioration. To the best of our knowledge, this is the first reported case of nephrotic syndrome due to MN in a patient with ADPKD treated with MFM, with remission of proteinuria and preservation of renal function after more than 10 years. Findings in this patient also suggest that MFM might reduce cystic cell proliferation and fibrosis, preventing progressive renal scarring with preservation of renal function. |
| format | Article |
| id | doaj-art-698a7c4651c0403b8f6f4200647bbbf0 |
| institution | Kabale University |
| issn | 1537-744X |
| language | English |
| publishDate | 2011-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | The Scientific World Journal |
| spelling | doaj-art-698a7c4651c0403b8f6f4200647bbbf02025-02-03T05:51:57ZengWileyThe Scientific World Journal1537-744X2011-01-01111041104710.1100/tsw.2011.94Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney DiseaseRamón Peces0Jorge Martínez-Ara1Carlos Peces2Mariluz Picazo3Emilio Cuesta-López4Cristina Vega5Sebastián Azorín6Rafael Selgas7Servicio de Nefrología, Hospital Universitario La Paz, IdiPaz, Madrid, SpainServicio de Nefrología, Hospital Universitario La Paz, IdiPaz, Madrid, SpainArea de Tecnologías de la Información, SESCAM, Toledo, SpainServicio de Anatomía Patológica, Hospital Universitario La Paz, Madrid, SpainServicio de Radiología, Hospital Universitario La Paz, Madrid, SpainServicio de Nefrología, Hospital Universitario La Paz, IdiPaz, Madrid, SpainServicio de Nefrología, Hospital Universitario La Paz, IdiPaz, Madrid, SpainServicio de Nefrología, Hospital Universitario La Paz, IdiPaz, Madrid, SpainWe report the case of a 38-year-old male with autosomal-dominant polycystic kidney disease (ADPKD) and concomitant nephrotic syndrome secondary to membranous nephropathy (MN). A 3-month course of prednisone 60 mg daily and losartan 100 mg daily resulted in resistance. Treatment with chlorambucil 0.2 mg/kg daily, low-dose prednisone, plus an angiotensin-converting enzyme inhibitor (ACEI) and an angiotensin II receptor blocker (ARB) for 6 weeks resulted in partial remission of his nephrotic syndrome for a duration of 10 months. After relapse of the nephrotic syndrome, a 13-month course of mycophenolate mofetil (MFM) 2 g daily and low-dose prednisone produced complete remission for 44 months. After a new relapse, a second 24-month course of MFM and low-dose prednisone produced partial to complete remission of proteinuria with preservation of renal function. Thirty-six months after MFM withdrawal, complete remission of nephrotic-range proteinuria was maintained and renal function was preserved. This case supports the idea that renal biopsy is needed for ADPKD patients with nephrotic-range proteinuria in order to exclude coexisting glomerular disease and for appropriate treatment/prevention of renal function deterioration. To the best of our knowledge, this is the first reported case of nephrotic syndrome due to MN in a patient with ADPKD treated with MFM, with remission of proteinuria and preservation of renal function after more than 10 years. Findings in this patient also suggest that MFM might reduce cystic cell proliferation and fibrosis, preventing progressive renal scarring with preservation of renal function.http://dx.doi.org/10.1100/tsw.2011.94 |
| spellingShingle | Ramón Peces Jorge Martínez-Ara Carlos Peces Mariluz Picazo Emilio Cuesta-López Cristina Vega Sebastián Azorín Rafael Selgas Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney Disease The Scientific World Journal |
| title | Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney Disease |
| title_full | Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney Disease |
| title_fullStr | Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney Disease |
| title_full_unstemmed | Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney Disease |
| title_short | Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney Disease |
| title_sort | nephrotic syndrome and idiopathic membranous nephropathy associated with autosomal dominant polycystic kidney disease |
| url | http://dx.doi.org/10.1100/tsw.2011.94 |
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