Visual impairment caused by retinal abnormalities in mesangiocapillary (membranoproliferative) glomerulonephritis type II ("dense deposit disease").
Patients with mesangiocapillary glomerulonephritis (MCGN) type II usually present by early adulthood with hematuria, proteinuria, and renal impairment, and these features often are accompanied by a partial lipodystrophy and an autoantibody for the alternative complement pathway convertase (C3NeF). The diagnosis of MCGN type II depends on the demonstration of "dense deposits" in the glomerular basement membrane (GBM). Most patients also have multiple subretinal white spots or drusen that are histopathologically identical with the GBM deposits and evident ophthalmoscopically by the time renal failure develops. Initially visual acuity and visual fields are preserved, but fluorescein angiography and specialized tests of retinal function, such as dark adaptation, electroretinography, and electrooculography, may be abnormal and will worsen progressively. Over the next 20 years, vision often deteriorates because of retinal atrophy, and sometimes because of subretinal neovascular membranes, macular detachment, and central serous retinopathy. The authors describe a patient with MCGN type II who presented with renal failure and impaired vision at the age of 59. He already had widespread retinal atrophy, and subsequently a subretinal membrane developed. The drusen seen in MCGN type II, like the partial lipodystrophy, are a helpful clinical pointer to the diagnosis of this condition. All patients with MCGN type II should be warned of the risk of retinal complications and reviewed by an ophthalmologist at presentation and regularly after about 10 years to minimize the loss of visual acuity from complications of the retinopathy.
Colville
Deb
D
Centre for Eye Research, University Department of Ophthalmology, Royal Victorian Eye and Ear Hospital, East Melbourne, Australia.
Guymer
Robyn
R
Sinclair
Roger A
RA
Savige
Judy
J
D001284:Atrophy; D001327:Autoimmune Diseases / Q000150:complications*; D018450:Disease Progression; D015432:Glomerulonephritis, Membranoproliferative / Q000145:classification / Q000150:complications*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012164:Retinal Diseases / Q000209:etiology*; D015593:Retinal Drusen / Q000209:etiology