title: Treatment options for C3 glomerulopathy.
abstract: OBJECTIVE
  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).
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors: Departments of Internal Medicine and Pediatrics, University of Iowa, Iowa City, Iowa, USA. carla-nester@uiowa.edu
authors: Carla M
authors: CM
authors: Nester
authors: Richard J
authors: RJ
authors: Smith
mesh_terms: D000293:Adolescent; D000328:Adult; D000818:Animals; D003167:Complement Activation / Q000187:drug effects*; D003176:Complement C3 / Q000378:metabolism*; D003170:Complement Pathway, Alternative / Q000187:drug effects; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D007668:Kidney / Q000187:drug effects* / Q000276:immunology / Q000473:pathology; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D003176:Complement C3; D007166:Immunosuppressive Agents