Clinicopathological features of idiopathic membranous nephropathy combined with IgA nephropathy: a retrospective analysi

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Clinicopathological features of idiopathic membranous nephropathy combined with IgA nephropathy: a retrospective analysis of 9 cases Ruimin Hu1,2, Guolan Xing2*, Huijuan Wu1 and Zhigang Zhang1*

Abstract Background: The concomitant presence of idiopathic membranous nephropathy and IgA nephropathy is rare. Here, we report 9 cases of phospholipase-A2-receptor (PLA2R) positive idiopathic membranous nephritis combined with IgA nephropathy, while reviewing publications regarding the pathological characteristics of this glomerolonephritis complication. Case presentation: Nine cases of renal biopsy tissues were retrospectively reviewed, including the clinicopathological features, the results of the immunofluorescence assays, and the electron microscopic examination. The patients mainly presented proteinuria and microscopic hematuria, and the serum anti-PLA2R was detected as positive in all of the patients. Histologically, a wide thickening of the glomerular basement membrane was observed in each of the 9 cases. Additionally, there existed mild hyperplasia in the mesangial cell and the matrix of the mesangial area. Immunofluorescence assays showed prominent glomerular granular staining on the glomerular capillary loops for IgG (++/+++), IgG4 (++/++++), and PLA2R (+/++). In addition, moderate IgA positive stains were focally or sparsely limited to the mesangial areas. Electron microscopy revealed subepithelial and mesangial electron-dense deposits. Conclusions: The results from the case analyses indicated that idiopathic membranous nephropathy combined with IgA nephropathy possess the clinicopathological features found in both components. It is suggested that serum anti-PLA2R and tissue PLA2R are important biomarkers that can assist in the diagnosis of idiopathic membranous nephropathy associated with IgA nephropathy. Keywords: Idiopathic membranous nephropathy, IgA nephropathy, PLA2R

Background Membranous nephropathy (MN) and IgA nephropathy (IgAN) are two distinct glomerular diseases. The most common cause of nephrotic syndrome in adults can be attributed to MN, in which approximately 70 % of this disease can be categorized as idiopathic MN (also referred to as primary MN). In Chinese as well as other * Correspondence: [email protected]; [email protected] 2 Department of Nephrology, The First Affiliated Hospital of Zhengzhou University, 1, Jianshe Road East, Erqi District, Zhengzhou, Henan 450052, People’s Republic of China 1 Department of Pathology, School of Basic Medical Sciences, Fudan University, 138, Yixueyuan Road, Xuhui District, Shanghai 200032, People’s Republic of China

Asian populations, IgAN has become a major pathological type of glomerular disease. Furthermore, it was estimated that IgAN accounts for 45.2 to 58.2 % of primary glomerular diseases in China [1–3]. Recently, an increasing number of reports have emerged in regards to IgAN combined with other renal diseases [1, 4]. However, studies involving the concomitant presence of idiopathic MN and IgAN are rare. The few reports availa