Nephrotic syndrome and idiopathic membranous nephropathy associated with autosomal-dominant polycystic kidney disease
Entity
UAM. Departamento de Medicina; Instituto de Investigación Sanitaria Hospital Universitario de La Paz (IdiPAZ)Publisher
Hindawi Publishing CorporationDate
2011-05-05Citation
10.1100/tsw.2011.94
The Scientific World Journal 11 (2011): 1041–1047
ISSN
2356-6140 (print); 1537-744X (online)DOI
10.1100/tsw.2011.94Funded by
This work has been supported in part by grants from the Instituto de Salud Carlos III from Ministerio de Ciencia e Innovación (EC08/00236) and Programa Intensificación Actividad Investigadora (Agencia Laín-Entralgo/CM) to R.P.Project
Gobierno de España. EC08/00236Editor's Version
http://dx.doi.org/10.1100/tsw.2011.94Subjects
ADPKD; Glomerulonephritis; Membranous nephropathy; Mycophenolate mofetil; Nephrotic syndrome; Proteinuria; MedicinaRights
© 2011 with authorAbstract
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
Files in this item
Google Scholar:Peces, Ramón
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Martínez-Ara, Jorge
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Peces, Carlos
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Picazo, Mariluz
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Cuesta-López, Emilio
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Vega, Cristina
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Azorín, Sebastián
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Selgas, Rafael
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