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Kidney Week

Abstract: TH-PO114

Sjogren Syndrome Manifesting as Thrombotic Microangiopathy and Cryoglobulinemic Vasculitis

Session Information

  • AKI: Mechanisms - I
    November 03, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
    Abstract Time: 10:00 AM - 12:00 PM

Category: Acute Kidney Injury

  • 103 AKI: Mechanisms

Authors

  • Kariyil, Reshma Joseph, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Haq, Kanza, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Dasgupta, Alana, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Bagnasco, S.M., Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Gautam, Samir Chandra, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
Introduction

Cryoglobulinemic vasculitis is an immune complex mediated small vessel vasculitis with significant morbidity and mortality. Renal manifestations are generally MPGN pattern but less commonly membranoproliferative glomerulopathy and intraglomerular thrombi.

Case Description

We present the case of a 49-year-old Palestinian female with history of SLE on hydroxychloroquine, hypertension, and chronic kidney disease with prior biopsy proven chronic thrombotic microangiopathy. She presented with dyspnea on exertion, lower extremity purpuric rash and edema, and new left foot weakness and numbness. Laboratory testing showed severe acute kidney injury, proteinuria as well as predominantly low C4 hypocomplementemia, positive ANA, RF, and anti-Ro52 and Ro62 antibodies. Renal biopsy showed rare glomeruli with thickened capillary walls and PAS-positive intracapillary coagula. Similar coagula were seen to fill the lumens of several small arteries (Fig 1, PAS 400x). These coagula stained brightly for IgM, IgG, C3, kappa, and lambda on immunofluorescence. On electron microscopy, rare cellular interpositioning with scattered paramesangial and intracapillary electron dense deposits were seen (Fig 2). These deposits were comprised of haphazardly arranged tubulocylinders with an average diameter of 27 nm. Additional laboratory testing revealed circulating type II cryoglobulin with high cryocrit > 40%. She was eventually diagnosed with cryoglobulinemic vasculitis and TMA from Sjogren’s syndrome. She was given steroids, rituximab, and ultimately required dialysis.

Discussion

Cryoglobulinemic vasculitis with renal involvement is most commonly seen with hepatitis C infection. Among renal manifestations, membranoproliferative pattern is common. Though our patient had fibrinoid necrosis of small arteries, glomerular thrombi were not present. Although association with Sjogren’s syndrome is rare, it is associated with higher risk of lymphoma and high systemic disease activity.