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Abstract: PO1785

Phospholipase A2 Receptor (PLA2R) Positive Membranous Nephropathy (MN) in Celiac Disease

Session Information

Category: Trainee Case Report

  • 1202 Glomerular Diseases: Immunology and Inflammation

Author

  • Muaddi, Luba, Allegheny General Hospital - Western Pennsylvania Hospital Medical Education Consortium, Pittsburgh, Pennsylvania, United States

Group or Team Name

  • AGH Nephrology Fellowship
Introduction

In a 2011 study, PLA2R antibody (Ab) was detected in lupus MN, HBV MN, and solid tumor associated MN, with IgG4 stained glomeruli. PLA2R+ patients did not achieve remission with HBV treatment and tumor resection, suggesting a coincidental occurrence of primary membranous nephropathy (PMN). PLA2R Ab levels in cases of PMN were higher (87.5%) than non-membrane nephropathy (0%) in a 2018 study. PLA2R was positive in 40 cases of secondary membranous nephropathy SMN (25%), including lupus MN, HBV MN, and atypical MN. In 2014, a case of celiac disease (CD) and H. Pylori infection with PLA2R+ MN was reported. Remission was achieved by H Pylori eradication without immunosuppression. In 2002, 2007 and 2009, three cases of renal failure due to MN in CD patients were reported raising the possibility of a link between the two conditions. We report the fourth case on the association of CD and MN to date.

Case Description

40-year-old male with iron deficiency anemia and small bowel biopsy proven CD presented with pleural effusions, hypoxia, and generalized anasarca. Infectious and rheumatologic work up was negative. Lung biopsy showed hemosiderosis reaching diagnosis of Lane-Hamilton Syndrome (LHS) (idiopathic pulmonary hemosiderosis (IPH), CD, chronic anemia). Three years prior, he was diagnosed with PMN by PLA2R+ renal biopsy and had nephrotic range proteinuria >9g. He was treated with losartan and diuretics with improvement in his symptoms and decrease in proteinuria to 6g. Serum creatinine rose from baseline of 1 to 1.9 mg/dL. He was then started on high dose prednisone for IPH and cyclosporine for MN with further improvement in proteinuria to 2.6g and creatinine to 1.3 mg/dL. Anti-thrombospondin type I domain-containing 7A Ab was negative arguing against PMN. He had been trying to adhere to gluten free diet but was not consistent.

Discussion

CD is known to cause IPH and chronic anemia. It was hypothesized that chronic gastrointestinal inflammation triggers auto antibody formation against PLA2R1, which is present in duodenal and gastric cells in addition to glomerular cells. This would favor a causal relationship rather than coincidence of two idiopathic processes. Gluten free diet and steroids are the mainstay of therapy for LHS. We hope to prove that adherence to strict gluten free diet in our patient in addition to sustained low dose prednisone would lead to remission of MN without need for cyclosporine.