title: Membranoproliferative glomerulonephritis type II (dense deposit disease): an update.
abstract: Membranoproliferative glomerulonephritis type II (MPGN II) is a rare disease characterized by the deposition of abnormal electron-dense material within the glomerular basement membrane of the kidney and often within Bruch's membrane in the eye. The diagnosis is made in most patients between the ages of 5 and 15 yr, and within 10 yr, approximately half progress to end-stage renal disease, occasionally with the late comorbidity of visual impairment. The pathophysiologic basis of MPGN II is associated with the uncontrolled systemic activation of the alternative pathway (AP) of the complement cascade. In most patients, loss of complement regulation is caused by C3 nephritic factor, an autoantibody directed against the C3 convertase of the AP, but in some patients, mutations in the factor H gene have been identified. For the latter patients, plasma replacement therapy prevents renal failure, but for the majority of patients, there is no proven effective treatment. The disease recurs in virtually all renal allografts, and a high percentage of these ultimately fail. The development of molecular diagnostic tools and new therapies directed at controlling the AP of the complement cascade either locally in the kidney or at the systemic level may lead to effective treatments for MPGN II.
authors.affiliation: Columbia University, Department of Nephrology, New York, New York, USA.
authors.forename: Gerald B
authors.initials: GB
authors.lastname: Appel
authors.forename: H Terence
authors.initials: HT
authors.lastname: Cook
authors.forename: Gregory
authors.initials: G
authors.lastname: Hageman
authors.forename: J Charles
authors.initials: JC
authors.lastname: Jennette
authors.forename: Michael
authors.initials: M
authors.lastname: Kashgarian
authors.forename: Michael
authors.initials: M
authors.lastname: Kirschfink
authors.forename: John D
authors.initials: JD
authors.lastname: Lambris
authors.forename: Lynne
authors.initials: L
authors.lastname: Lanning
authors.forename: Hans U
authors.initials: HU
authors.lastname: Lutz
authors.forename: Seppo
authors.initials: S
authors.lastname: Meri
authors.forename: Noel R
authors.initials: NR
authors.lastname: Rose
authors.forename: David J
authors.initials: DJ
authors.lastname: Salant
authors.forename: Sanjeev
authors.initials: S
authors.lastname: Sethi
authors.forename: Richard J H
authors.initials: RJ
authors.lastname: Smith
authors.forename: William
authors.initials: W
authors.lastname: Smoyer
authors.forename: Hope F
authors.initials: HF
authors.lastname: Tully
authors.forename: Sean P
authors.initials: SP
authors.lastname: Tully
authors.forename: Patrick
authors.initials: P
authors.lastname: Walker
authors.forename: Michael
authors.initials: M
authors.lastname: Welsh
authors.forename: Reinhard
authors.initials: R
authors.lastname: Würzner
authors.forename: Peter F
authors.initials: PF
authors.lastname: Zipfel
mesh_terms: D002648:Child; D015432:Glomerulonephritis, Membranoproliferative / Q000473:pathology* / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D007678:Kidney Glomerulus / Q000473:pathology; D012160:Retina / Q000473:pathology; D012164:Retinal Diseases / Q000473:pathology / Q000503:physiopathology