Complement inhibition in C3 glomerulopathy

Highlights • Dysregulation of the alternative pathway of complement plays a critical role in C3 glomerulopathy. • Ultra-rare variants in complement genes are identified in about 40% of C3 glomerulopathy patients. • C3 nephritic factors are present in the majority of patients with C3 glomerulopathy....

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Veröffentlicht in:	Seminars in immunology 2016-06, Vol.28 (3), p.241-249
Hauptverfasser:	Nester, Carla M, Smith, Richard J.H
Format:	Artikel
Sprache:	eng
Schlagworte:	Allergy and Immunology Animals Biomarkers - metabolism C3 glomerulonephritis C3 glomerulopathy Complement Complement C3 - genetics Complement C3 - immunology Complement Inactivating Agents - therapeutic use Complement Pathway, Alternative Dense deposit disease Eculizumab Gene Frequency Genotype Glomerulonephritis, Membranous - genetics Glomerulonephritis, Membranous - immunology Glomerulonephritis, Membranous - therapy Humans Immunotherapy - methods Kidney - metabolism Kidney - pathology Membranoproliferative glomerulonephritis Polymorphism, Genetic
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Beschreibung
Zusammenfassung:	Highlights • Dysregulation of the alternative pathway of complement plays a critical role in C3 glomerulopathy. • Ultra-rare variants in complement genes are identified in about 40% of C3 glomerulopathy patients. • C3 nephritic factors are present in the majority of patients with C3 glomerulopathy. • Complement biomarker abnormalities are common in C3 glomerulopathy. • Complement inhibitors provide a targeted approach to the treatment of C3 glomerulopathy.
ISSN:	1044-5323 1096-3618
DOI:	10.1016/j.smim.2016.06.002