イトウ　ナオコ   Itou Naoko   井藤　奈央子    所属   医学部 医学科（東京女子医科大学病院）    職種   助教
論文種別	総説
言語種別	英語
査読の有無	査読あり
招待の有無	招待あり
表題	C3 glomerulopathy and current dilemmas.
掲載誌名	正式名：Clinical and experimental nephrology 略　 称：Clin Exp Nephrol ISSNコード：14377799/13421751
掲載区分	国外
巻・号・頁	21(4),pp.541-551
著者・共著者	Ito Naoko, Ohashi Ryuji, Nagata Michio
発行年月	2017/08
概要	C3 glomerulopathy (C3G) is a recently identified disease entity caused by dysregulation of the alternative complement pathway, and dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are its components. Because laboratory detection of complement dysregulation is still uncommon in practice, "dominant C3 deposition by two orders greater than that of immunoglobulins in the glomeruli by immunofluorescence", as stated in the consensus report, defines C3G. However, this morphological definition possibly includes the cases with glomerular diseases of different mechanisms such as post-infectious glomerulonephritis. In addition, the differential diagnosis between DDD and C3GN is often difficult because the distinction between these two diseases is based solely on electron microscopic features. Recent molecular and genetic advances provide information to characterize C3G. Some C3G cases are found with genetic abnormalities in complement regulatory factors, but majority of cases seem to be associated with acquired factors that dysregulate the alternative complement pathway. Because clinical courses and prognoses among glomerular diseases with dominant C3 deposition differ, further understanding the background mechanism, particularly complement dysregulation in C3G, is needed. This may resolve current dilemmas in practice and shed light on novel targeted therapies to remedy the dysregulated alternative complement pathway in C3G.
DOI	10.1007/s10157-016-1358-5
PMID	27878657