Multimodal imaging of retinal pigment epithelial detachments in patients with C3 glomerulopathy: case report and review of the literature

Abstract

BackgroundTo describe the optical coherence tomography angiograhy (OCTA) of drusenoid pigment epithelial detachments (PEDs) in a woman affected by Complement 3 (C3) glomerulopathy, which represents a spectrum of glomerular diseases characterized on fluorescent microscopy by C3 accumulation with absent, or scanty, immunoglobulin deposits. It is due to acquired or genetically defective alternative pathway control and is generally associated with drusen-like deposits in Bruch’s membrane, as well as choriocapillaris. These retinal lesions can be associated with choroidal neovascularization and central serous chorioretinopathy (CSCR). OCTA is useful to detect neovascularization without injecting a contrast product, particularly in these patients who may have renal insufficiency.Case presentationA 28-year-old woman affected by C3 glomerulpathy was diagnosed with asymptomatic multiple bilateral PEDs during a routine ophthalmologic consultation. To better characterize the lesions, multimodal imaging was performed and included: optic coherence tomography (OCT), en-face OCT, OCTA, fluorescence and indocyanine angiography. The OCTA clearly identified vascular network rarefaction with decreased choriocapillary vascularization. It confirmed that PEDs associated with C3 glomerulonephritis are not vascularized, but rather of serous type.ConclusionsPatients affected by C3 glomerulopathy can develop neovascular membranes as retinal complications of pigment epithelial detachments. Optical coherence angiography may be indicated to identify this complication, without injecting any contrast product that could produce further kidney damage.

Progressive chorioretinal involvement in a patient with light-chain (AL) amyloidosis: a case report

“…These features were distinct from pattern dystrophy and Stargardt disease that usually do not manifest with subretinal fluid. The subretinal deposits observed in this case are different from those found in the membranoproliferative glomerulonephritis [13,14]. The pachychoroid feature with a widened choriocapillaris was first outlined by Roybal [11] and may be secondary to light chain deposition as suggested in several postmortem histological studies [15,16].…”

Increased risk for central serous chorioretinopathy in nephrotic syndrome patients: A population-based cohort study

“…19 Another glomerular disease, membranoproliferative glomerulonephritis (MPGN) type II, which could sometimes present with NS, has also been repeatedly reported to be associated with CSCR in several case reports. [19][20][21][22] MPGN type II, also referred to as dense deposit disease, is characterized by electron-dense deposits in the glomerular basement membrane (GBM). In a new classification of MPGN using immunofluorescence microscopy, MPGN type II is classified under complement 3 (C3) glomerulopathies, which is a term encompassing all 3 types of MPGN with immune complex-mediated and complement-mediated mechanisms, and it is characterized by C3 deposits in the mesangium, subendothelial, and subepithelial capillary walls of the glomerulus.…”

“…[15][16][17][18][19] Besides, membranoproliferative glomerulonephritis type II, one of the common NS, 20 has been repeatedly described in the literature to be characterized by serous retinal detachment and subretinal deposits, resembling classical CSCR. [20][21][22] Moreover, the use of corticosteroids, which have been commonly used to treat NSs, 23,24 is also a well-known risk factor for CSCR. 25,26 Despite these numerous reported associations between NS and CSCR in the literature, there is no epidemiologic evidence linking them together.…”