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Treatment options for C3 glomerulopathy

Nester, Carla M.a,c; Smith, Richard J.a,b,c

Current Opinion in Nephrology and Hypertension: March 2013 - Volume 22 - Issue 2 - p 231–237
doi: 10.1097/MNH.0b013e32835da24c
CLINICAL NEPHROLOGY: Edited by Bradley M. Denker

Purpose of review The purpose of this review is to discuss emerging nomenclature, review the salient clinicopathological features and describe the therapeutic options available for the treatment of C3 glomerulopathy (C3G).

Recent findings C3G is minimally responsive to traditional immune suppression and randomized controlled trials to support therapy are absent. The burgeoning understanding of the role of the alternative complement pathway in C3G combined with animal data supporting the use of terminal complement blockade and a few reports suggesting that the anticomplement drug eculizumab may offer a therapeutic advantage have triggered great interest in the field of complement-mediated renal disease.

Summary Anticellular immune suppression and plasma therapy have limited efficacy in C3G. Data suggest that eculizumab may ameliorate disease in some C3G patients. The limited, recently published cohort data highlight crucial aspects of this group of diseases and support the need for extensive genetic and biomarker research to validate the pathologic mechanisms, delineate the spectrum of disease and guide the design of the rigorous trials to identify effective therapies for the treatment of C3G.

aDepartments of Internal Medicine and Pediatrics

bIowa Institute of Human Genetics

cRare Renal Diseases Clinic, University of Iowa, Iowa City, Iowa, USA

Correspondence to Carla M. Nester, MD, MSA, Rare Renal Diseases Clinic, University of Iowa Hospitals and Clinics 200 Hawkins Drive, BT 4036, Iowa City, IA 52242-1081, USA. Tel: +1 319 353 7335; e-mail:

© 2013 Lippincott Williams & Wilkins, Inc.