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Abstract: PO1790

A Unique Case of Autoimmune-Mediated Cryoglobulinemic Glomerulonephritis

Session Information

Category: Trainee Case Report

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Sadlak, Monika, TriHealth, Cincinnati, Ohio, United States
  • Muneer, Sumayya, TriHealth, Cincinnati, Ohio, United States
  • Rajput, Amit K., TriHealth, Cincinnati, Ohio, United States
Introduction

Cryoglobulinemic syndrome is a disease in which immunoglobulin components are deposited within tissues, resulting in various end-organ damage. Subtypes Type II and III contain mixed monoclonal and polyclonal immunoglobulins, thereby referred to as Mixed Cryoglobulin Syndrome (MC). MC is often associated with infections such as Hepatitis C; however, it can also be secondary to autoimmune diseases. While the most common associations are with Systemic Lupus Erythematosus and Sjogren’s disease, occasionally, MC can be seen with other rheumatologic conditions. We examine a rare case of Overlap Syndrome (OS) induced cryoglobulinemic glomerulonephritis (CG).

Case Description

A 44-year-old woman with a history of OS, presented with symptoms of fatigue, generalized edema, weight gain of 20lbs, and found to have an acute kidney injury (AKI). She was admitted to the hospital with a diagnosis of decompensated heart failure. The diagnosis of OS was confirmed with elevated ANA, SSA, RNP antibodies, as well as negative dsDNA and anti-smith antibodies. Previously, she was unsuccessfully treated with methotrexate and hydroxychloroquine. She was on hydroxychloroquine monotherapy at the time of admission.

During the investigation for her AKI, she was found to have hematuria and non-nephrotic range proteinuria (UPCR 2.65g/dL), raising concerns for glomerulonephritis. Subsequent renal biopsy showed autoimmune-mediated cryoglobulinemic glomerulonephritis. She was treated with a combination of methylprednisolone/prednisone and rituximab, which resulted in normalization of renal function.

Discussion

This case illustrates a patient with a history of OS with biopsy proven autoimmune mediated CG. The patient’s underlying diagnosis of OS is likely the leading risk factor for renal impairment secondary to immunoglobulin deposits. Treatment is focused on immunosuppression, including steroids, rituximab, or mycophenolate mofetil. Combination therapy with a non-steroid immunosuppressant is preferred over monotherapy with steroids. The patient was treated with steroids and rituximab, with recovery of renal function. Given the varying presentation of CG, physicians should be mindful of keeping a broad differential, particularly in patients with rheumatological history presenting with proteinuria, hematuria, and signs of renal dysfunction.