Abstract
Purpose: To determine the retinal features of membranoproliferative glomerulonephritis (MPGN) type 2(II) in a cohort of patients on the Island of Ireland and to correlate this with age of onset of retinal findings.
Methods: Thirty-six individuals were identified as having MPGN II from renal clinics in Belfast and Dublin, 21 from Northern Ireland and 15 from Republic of Ireland. Each individual had a full medical review of the histopathological diagnosis, fundus retinal imaging (a subset also had OCT) and were genotyped. Retinal images were graded by the Belfast Ophthalmic Reading Centre and a consultant ophthalmologist for any retinal findings. Drusen were graded as hard distinct, soft distinct, soft indistinct and reticular (table 1) and within the regions cone-temporal, generalised and macular. All data were collated into an excel spreadsheet and statistical analysis was conducted.
Results: Of the 36, 28 (77.8%) had fundus images and 6 (16.7%) had OCT. The mean age was 67.6 years, and the average age of onset was 17 years. There were 23 females (63.9%) and 13 males (36.1%). Some, 25% had had more than 1 transplant. Only 2 described visual symptoms, 1 diplopia and 1 macular dystrophy.
Nearly all, 98% showed various degrees of RPE atrophy and 100% of individuals showed disease involvement in the macula region. A majority, 68% showed one or more categories of drusen with 44% showing soft indistinct drusen. The most common type of distribution was cone-temporal (34%). Only 9% of individuals showed evidence of fibrosis. Some, 30% showed some degree of optic disc cupping but only 4% showed optic disc oedema. In addition, 42% showed pigmentary changes. On OCT, 27% of all cases had an epi-retinal membrane, of these 56% lacked the presence of drusen.
Conclusions: All individuals with MPGN II have shown some degree of retinal involvement. Generalised atrophy in the RPE is the most common finding. The majority of cases have shown lipoid depositions in the form of soft indistinct drusen that are distributed in a cone like configuration towards the temporal end of the eye globe. However other features such as fibrosis and pigmentary changes have been observed at a relatively lower percentage.
Methods: Thirty-six individuals were identified as having MPGN II from renal clinics in Belfast and Dublin, 21 from Northern Ireland and 15 from Republic of Ireland. Each individual had a full medical review of the histopathological diagnosis, fundus retinal imaging (a subset also had OCT) and were genotyped. Retinal images were graded by the Belfast Ophthalmic Reading Centre and a consultant ophthalmologist for any retinal findings. Drusen were graded as hard distinct, soft distinct, soft indistinct and reticular (table 1) and within the regions cone-temporal, generalised and macular. All data were collated into an excel spreadsheet and statistical analysis was conducted.
Results: Of the 36, 28 (77.8%) had fundus images and 6 (16.7%) had OCT. The mean age was 67.6 years, and the average age of onset was 17 years. There were 23 females (63.9%) and 13 males (36.1%). Some, 25% had had more than 1 transplant. Only 2 described visual symptoms, 1 diplopia and 1 macular dystrophy.
Nearly all, 98% showed various degrees of RPE atrophy and 100% of individuals showed disease involvement in the macula region. A majority, 68% showed one or more categories of drusen with 44% showing soft indistinct drusen. The most common type of distribution was cone-temporal (34%). Only 9% of individuals showed evidence of fibrosis. Some, 30% showed some degree of optic disc cupping but only 4% showed optic disc oedema. In addition, 42% showed pigmentary changes. On OCT, 27% of all cases had an epi-retinal membrane, of these 56% lacked the presence of drusen.
Conclusions: All individuals with MPGN II have shown some degree of retinal involvement. Generalised atrophy in the RPE is the most common finding. The majority of cases have shown lipoid depositions in the form of soft indistinct drusen that are distributed in a cone like configuration towards the temporal end of the eye globe. However other features such as fibrosis and pigmentary changes have been observed at a relatively lower percentage.
| Original language | English |
|---|---|
| Publication status | Published - 05 May 2024 |
| Event | Association for Research in Vision and Ophthalmology Annual Meeting 2024 - Seattle, United States Duration: 05 May 2024 → 09 May 2024 |
Conference
| Conference | Association for Research in Vision and Ophthalmology Annual Meeting 2024 |
|---|---|
| Abbreviated title | ARVO 2024 |
| Country/Territory | United States |
| City | Seattle |
| Period | 05/05/2024 → 09/05/2024 |
Keywords
- kidney
- dense deposit disease
- retinal changes
- imaging
- Rare disease