Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney Disease

Abstract

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.

Co-existing autosomal dominant polycystic kidney disease and nephrotic syndrome in a Nigerian patient with lupus nephritis

“…Among them, three renal histology (37.5%) showed lupus nephritis while others showed each of idiopathic membranous glomerulonephritis, diffuse proliferative glomerulonephritis, amyloidosis, mesangioproliferative glomerulonephritis, and focal segmental glomerulosclerosis. [ 4 5 6 7 8 9 10 ]…”

Myeloperoxidase-antineutrophil cytoplasmic antibody-associated crescentic glomerulonephritis in autosomal dominant polycystic kidney disease

“…Among these 17 patients, 4 had FSGS and this represented the most common histological subtype associated with ADPKD [3-6], followed by 3 cases of membranous nephropathy [7-9], 2 cases each of minimal change disease [10,11], IgA nephropathy [12,13], and post-infectious mesangial proliferative glomerulonephritis [14,15], and one case each of membranoproliferative glomerulonephritis [15], mesangioproliferative glomerulonephritis [16], diabetic glomerulosclerosis [17], and crescentic glomerulonephritis [18]. The high incidence of FSGS suggests that glomerular hyperfiltration could play an important role in the development of FSGS and heavy proteinuria in ADPKD patients, while FSGS may in turn be important for progression to ESRD in a subgroup of ADPKD patients [3].…”

Acute Kidney Injury due to Crescentic Glomerulonephritis in a Patient with Polycystic Kidney Disease

“…However, when this has been reported, various etiologies have been identified, including membranous glomerulonephritis, focal and segmental glomerulosclerosis, minimal change disease [8, 9, 10] and immunoglobulin A (IgA) nephropathy [11, 12]. Of these, focal and segmental glomerulosclerosis appears to be the most frequently reported cause of nephrotic-range proteinuria in those with ADPKD who develop proteinuria [8, 13].…”