02001nas a2200217   4500000000100000008004100001100001900042700001200061700001200073700001200085700001100097700001100108700001200119700001400131700001400145700001200159700001400171245006500185520151900250022001401769       2018                            d1 aRamachandran R1 aKohli H1 aGupta K1 aRathi M1 aNada R1 aJha V.1 aYadav A1 aInamdar N1 aBharati J1 aKumar A1 aPrakash G00aMembranous Nephropathy With Light Chain Restricted Deposits.3 a<p>
The literature on Membranous nephropathy (MN) with monoclonal deposits on immunofluorescence (IF) and their outcome is very scarce. We report our experience of managing five patients with this clinical entity. Mean age of the patients was 33.2±6.55 years. The mean proteinuria, serum albumin and serum creatinine was 5.73±2.17 g/day, 2.86±0.51 g/dL and 1.34±1.19 mg/dL, respectively. None of the patients had a lymphoproliferative disorder. Only one patient had an elevated free light chain ratio. Four (80%) patients were M-type phospholipase A2 receptor (PLA2R) negative (tissue and serum), and 1 (20%) was PLA2R related. Three (60%) cases had monoclonal IgG3/k, one IgG3/λ, whereas one patient with PLA2R positivity had an IgG3/IgG4k subtype. Two (67%) patients treated with cyclical cyclophosphamide and steroids (cCYC/GC) achieved complete remission and one patient (33%) with elevated baseline creatinine had a reduction in serum creatinine with persistent proteinuria at the end of 12th month of follow-up. One patient with PLA2R positive MN was treated with Rituximab and is in complete remission. The patient with an elevated free light chain at baseline was treated with Bortezomib/Thalidomide/Dexamethasone, had complete remission at 12 months, however, had a progressive rise in creatinine over the next 40 months of follow-up. The current series though limited by numbers documents the efficacy of conventional therapies in non-malignant associated MN with monoclonal deposits on IF.
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