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Abstract: TH-PO0786

A Rare Membranoproliferative Glomerulonephritis Lesion: Cell-Mediated Without Immune Deposits

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Churilla, Bryce M, Emory University School of Medicine, Atlanta, Georgia, United States
  • Caberto, Sheryl Cababat, Emory University School of Medicine, Atlanta, Georgia, United States
Introduction

Membranoproliferative glomerulonephritis (MPGN) is characterized by a pattern of mesangial and endocapillary hypercellularity with duplication of the glomerular basement membrane. Immune complex deposit or complement-mediated injury is usually evident in immunofluorescence (IF) staining. However, in exceedingly rare instances, the renal damage can be cell mediated.

Case Description

Patient is a 61-year-old female veteran, with history of non-ischemic cardiomyopathy, status post heart transplant in 2016, and chronic kidney disease stage 3 which was presumed secondary to calcineurin-inhibitor, presented for acute onset of nephrotic syndrome and pancytopenia. Initial workup was remarkable for proteinuria with urine-protein-creatinine ratio 5.6 g/g. Serologic tests were negative for ANA, dsDNA, ANCA, anti-GBM, anti-PLAR2, complements, HIV and hepatitis. Lactate dehydrogenase was elevated and haptoglobin was low, but hemolysis was ruled out with low reticulocyte and normal bilirubin. Serum and urine electrophoresis were insignificant as well as free light chains. All workups were unrevealing; thus, patient underwent kidney biopsy with a finding of membranoproliferative pattern of glomerulonephritis without immune complexes.

Discussion

The kidney biopsy was notable for moderate-to-severe increase in mesangial cells. There was endocapillary hypercellularity which were predominantly T-cells and CD68-positive macrophages, as well as occasional polymorphonuclear neutrophils. There were no immune complexes noted. Hypercellularity was extensive which is not consistent with chronic thrombotic microangiopathy. Therefore, there was a concern for a cell-mediated injury, which is not well-defined in existing literature.

Patient’s clinical presentation was suspected to result from a T-cell mediated response, although the etiology remained unidentified. Steroid therapy, which targets T-cell cellular immune responses, was preferred in this case. Limited literature on this injury pattern shows variable responses to immunosuppression; however, the patient responded well to tapering dose of steroid.

This extremely rare lesion of MPGN may help identify common characteristics, kidney biopsy findings and treatment options in similar cases.

Digital Object Identifier (DOI)