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Kidney Week

Abstract: FR-PO679

AKI from Polytypic Plasma Cell Interstitial Infiltration in Sjogren Syndrome

Session Information

Category: Trainee Case Report

  • 1500 Onco-Nephrology

Authors

  • Javed, Muniba, University of Washington, Seattle, Washington, United States
  • Malik, A. Bilal, University of Washington, Seattle, Washington, United States
Introduction

57-year-old woman with a history of extensively treated Marginal Zone Lymphoma diagnosed with concomitant pulmonary AL Amyloidosis on lung biopsy in 2011 as well as remote history of Sjogren Syndrome, referred for acute kidney injury (AKI). She was last treated with rituximab for lymphoma 6 months ago.

Case Description

During initial evaluation (1/22/19), she was found to have a relatively brisk worsening of creatinine (Cr) from 0.9 to 2mg/dL over a 2- week period. She had new onset microscopic hematuria, glycosuria and pyuria with proteinuria quantified as 1.3g (albumin/cr of 66mg/g). Ultrasound showed edematous bilateral kidneys with serial CT imaging highlighting an insidiously worsening loss of corticomedullary differentiation over the last year. Ancillary studies showed a stably abnormal k/l ratio of 3.5 and IgG- k M- spike of 0.1g/dL, with these serologies showing chronic and fluctuating elevated titers since the diagnosis of Lymphoma. There was also a gradually worsening total serum protein of 11.1g/dL and IgG titer of 5925mg/dL (normal < 1616). Native kidney biopsy was undertaken on 2/1/19 that showed extensive interstitial infiltration with polytypic plasma cells with no morphologic or immunophenotypic evidence of lymphoma. Just prior to kidney biopsy, Cr peaked at 2.96mg/dL and prednisone 1 mg/kg was initiated for empiric management. Given absence of lymphomatous renal involvement, chemotherapy was not entertained. Renal function improved with Cr dropping to 0.7mg/dL following a tapering course of prednisone. This was accompanied by resolution of pyuria and stabilization of IgG titer to 3725mg/dL on last check. She tested positive for serum ANA (positive ss-A and ss-B) with negative double- stranded DNA.

Discussion

Patient experienced AKI from a non-lymphomatous polycloncal plasmacytic interstitial infiltrate concomitant with a severe hypergammaglobulinemia that responded briskly to prednisone. In the context of worsening sicca symptoms over the last 6 months and positive serologies, the plasmacytic infiltration was deemed a manifestation of Sjogren syndrome, which was first diagnosed in 2005 following biopsy of minor salivary glands. This presentation of renal failure from Sjogren syndrome is a rare occurrence and even more intriguing in a patient with extensive history of lymphoma. Accurate diagnosis in this case was essential to avoid unnecessary use of chemotherapy.