Colville Deb, Guymer Robyn, Sinclair Roger A, Savige Judy
Centre for Eye Research, University Department of Ophthalmology, Royal Victorian Eye and Ear Hospital, East Melbourne, Australia.
Am J Kidney Dis. 2003 Aug;42(2):E2-5. doi: 10.1016/s0272-6386(03)00665-6.
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.
II型系膜毛细血管性肾小球肾炎(MCGN)患者通常在成年早期出现血尿、蛋白尿和肾功能损害,这些特征常伴有部分脂肪营养不良以及针对替代补体途径转化酶的自身抗体(C3NeF)。II型MCGN的诊断取决于在肾小球基底膜(GBM)中发现“致密沉积物”。大多数患者还存在多个视网膜下白点或玻璃膜疣,其组织病理学与GBM沉积物相同,并且在肾衰竭发生时通过检眼镜检查可明显看到。最初视力和视野保持正常,但荧光素血管造影和视网膜功能的专门检查,如暗适应、视网膜电图和眼电图,可能会异常,并且会逐渐恶化。在接下来的20年里,视力通常会因视网膜萎缩而恶化,有时也会因视网膜下新生血管膜、黄斑脱离和中心性浆液性视网膜病变而恶化。作者描述了一名59岁的II型MCGN患者,其出现肾衰竭和视力受损。他已经有广泛的视网膜萎缩,随后出现了视网膜下膜。II型MCGN中出现的玻璃膜疣,与部分脂肪营养不良一样,是有助于诊断这种疾病的临床指标。所有II型MCGN患者都应被告知视网膜并发症的风险,并在就诊时由眼科医生进行检查,大约10年后定期复查,以尽量减少因视网膜病变并发症导致的视力丧失。