A case of glomerulopathy showing podocytic infolding in association with Sjögren’s syndrome and primary biliary cirrhosis

Abstract

A 49-year-old man was admitted to our hospital with mild proteinuria. Prior to admission, he had been diagnosed as having Sjögren's syndrome in association with primary biliary cirrhosis. Examination of a renal biopsy under light microscopy revealed diffuse and global mesangial cell proliferation and a spike and/or bubbling formation of the glomerular basement membrane (GBM), resembling membranoproliferative glomerulonephritis. In contrast, immunofluorescent studies showed marked immunoglobulin and complement depositions in the mesangial areas; however, only faint granular IgG and IgA deposition was observed along the GBM. Interestingly, electron microscopy revealed that a microtubular structure, derived from podocytes, was present in the GBM. We present a case of glomerulopathy showing podocytic infolding in association with Sjögren's syndrome and primary biliary cirrhosis.

Podocyte Infolding Glomerulopathy: A Special Morphology of Podocyte Injury Caused by Heterogeneous Diseases

“…The relatively higher incidence of IC-PIG is consistent with previous reports. 3 , 4 , 5 , 6 , 7 , 8 , 9 , 12 , 13 , 14 , 18 , 19 , 20 , 21 , 22 LMD/MS identified the protein ACTN4, which serves as a significant actin crosslinking cytoskeletal protein for maintaining normal podocyte structure, dramatically decreased than in the donor kidney group. Confocal microscope found that the ACTN4 and COL4A3 colocalized, which further verified that the GBM microstructures visualized in EM originated from the podocyte.…”

Nephrotic Syndrome due to Focal Segmental Glomerulosclerosis Complicating Sjögren’s Syndrome: A Case Report and Literature Review

“…Reportedly, renal amyloidosis [12], podocytic infolding glomerulopathy [13], lupus-associated lesions [4], and anti-neutrophil cytoplasmic antibody-associated nephritis are known to occur in patients with SjS [4]. Treatment for renal involvement primarily includes the administration of corticosteroid, and a few patients receive other immunosuppressants such as cyclosporine, cyclophosphamide, mycophenolate mofetil, and rituximab.…”

A Case of Podocytic Infolding Glomerulopathy with Diabetes Mellitus, Nephrotic Range Proteinuria and Positive Anti-PLA2R in Serum

“…Electron microscopy reveals infolding of the cytoplasm of the podocytes into the GBM. Most of the cases reported worldwide, indicate that PIG usually co-exists with another autoimmune disease [2,3]. In this case we present a diabetic patient with no other autoimmune disease in his medical history, whose biopsy was characterized as PIG.…”