Kidney Week

Abstract: PUB374

Complement 3 Glomerulopathy and Subsequent Atypical Hemolytic Uremic Syndrome

Session Information

• Publication Only

Category: Glomerular Diseases

• 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

• Nguyen, Christian, Texas Health Harris Methodist Hospital Fort Worth, Fort Worth, Texas, United States

Christian Nguyen, DO

• Schmidt, Brendan, Texas Health Harris Methodist Hospital Fort Worth, Fort Worth, Texas, United States

Brendan Schmidt, DO

• Nyalakonda, Ramyashree Rao, University of North Texas Health Science Center, Fort Worth, Texas, United States

Ramyashree Rao Nyalakonda

• Fairweather, Morgan G., Texas Health Harris Methodist Hospital Fort Worth, Fort Worth, Texas, United States

Morgan G. Fairweather, MD, MBA

• Housini, Ihsan, Texas Health Harris Methodist Hospital Fort Worth, Fort Worth, Texas, United States

Ihsan Housini

• Kanu, Obiajulu, UNC Health, Chapel Hill, North Carolina, United States

Obiajulu Kanu, MD

Introduction

Complement 3 glomerulopathy (C3G) is a collection of rare renal diseases that can be driven by genetic anomalies or, more commonly, acquired protein dysfunction that results in C3 deposition into the renal tissues. We present the case of an 80-year-old female with prior concerns of renal cell carcinoma that developed rapidly progressive renal dysfunction, progressing to dialysis and subsequently diagnosed with C3 glomerulonephritis (C3GN). Treatment was initiated with pulse-dose methylprednisolone, mycophenolate mofetil (MMF), and eculizumab – a novel treatment regimen relying on the augmentation of steroid and MMF treatment with eculizumab rather than transitioning therapy to eculizumab alone as previously described in severe cases of C3G. Interestingly, our patient later developed atypical hemolytic uremic syndrome (aHUS).

Case Description

An 80-year-old female with a medical history of diastolic heart failure and chronic kidney disease stage 3a returned to the emergency room after a previous admission for a complicated UTI. Her new complaints were shortness of breath, 20lbs of weight gain and productive cough.

Discussion

C3G is characterized by the deposition of C3 complement within the renal tissues due to a dysregulation of the C3 complement activation pathway. Another recently described phenomenon, aHUS, is also posited to be due to dysfunction of the alternate pathway of complement activation. While individually each syndrome is considered rare, the lack of literature may be due to limited reporting as opposed to non-occurrence coinciding with the novelty and relatively recent characterization of each disease process. This report adds to the growing literature describing the clinical course, diagnosis and treatment of C3G as well as C3G in relation to aHUS.

Ultrastructural study shows subepithelial and rare subendothelial deposits.