• Ali Jandal, Weixiong Zhong, Deepak Gopal, Vanessa Horner, Leah Frater-Rubsam, Arjang Djamali, and Gauri Bhutani.
• Divsion of Nephrology, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, United States.
• Am. J. Med. Sci. 2023 Mar 1; 365 (3): 286293286-293.

AbstractWe report a 36-year-old female with mixed nephritic-nephrotic syndrome and recurrent pancreatitis. Kidney biopsy showed a crescentic membranoproliferative glomerulonephritis with dominant C3 staining on immunofluorescence (IF) but only scant deposits on electron microscopy (EM) and instead, evidence of severe acute and chronic microangiopathy - endothelial swelling, sub-endothelial fluff, and segmental basement membrane remodeling. Her serum C3 was normal, Factor Ba, and serum Membrane attack complex (sMAC) levels were elevated, and Properdin was low. Genetic testing revealed a heterozygous ultra rare C3 variant of unknown significance (c.4838G>T, p.Gly1613Val) as well as a heterozygous deletion of CFHR3-CFHR1. She showed an initial response to terminal complement blockade with eculizumab, but her renal disease progressed in the next year. Notably, our patient never demonstrated microangiopathic hemolysis, yet pancreatitis of unclear etiology recurred periodically. Our case suggests the existence of a "C3G/aHUS overlap" clinicopathologic syndrome and highlights the challenges of treating complement-mediated kidney disease.Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.

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