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Age-related macular degeneration: diagnosis and management. London: National Institute for Health and Care Excellence (NICE); 2018 Jan. (NICE Guideline, No. 82.)
K.1. AMD overview and mapping of classification systems
| NICE recommended classification | NICE recommended classification | NICE recommended definitions | Modified International Criteria (NICE favoured classification system) | AREDS 9-step | AREDS 4 step | AREDS Simple Severity | Three continent | CARMS | Sandberg |
|---|---|---|---|---|---|---|---|---|---|
| Normal eyes | NA | Normal- no signs of AMD at all
or Small/hard drusen (<63 µm) only [MIC] | Normal- no signs of ARM at all
or Small/hard drusen (<63 µm) only [MIC] | Stage 1 | Stage 1 | Score of 0 (plus fellow eye) | No AMD | Grade 1 (if less than 10 small drusen) | Stage 1-2 (dependent on number of drusen) |
| Early AMD | NA |
|
| Stage 12 | Stage 2 | Score of 0 (plus fellow eye) | No AMD | Grade 2 | Stage 1-2 (dependent on number of drusen) |
| Score of 1 (plus fellow eye) | No AMD | Grade 2 | ungradable | |||||
|
| Stage 2-8 | Stage 3 | Score of 1 (plus fellow eye) | Mild early AMD | Grade 3 | Grade 3 | ||
| Score of 2 (plus fellow eye) | Mild early AMD | Grade 2 | Grade 2 (dependent on number) | |||||
|
| Score of 2 (plus fellow eye) | Moderatesevere early AMD | Grade 3 | Grade 3-4 | ||||
| |||||||||
| Late AMD | Indeterminate |
| chronic central serous chorioretinopathy [CSCR] | ||||||
| |||||||||
| Wet active
(the presence of serous or haemorrhagic retinal PED, a subretinal neovascular membrane, a subretinal haemorrhage, or a combination of the above-modified from [MIC], [AREDS4], [CARMS]) |
| Grade 4 | Stage 4 | Late AMD | Grade 5 | ||||
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| |||||||||
| Dry |
| Stage 9 | Stage 3 (extra foveal) and stage 4 | Late AMD | Grade 4 | ||||
| Wet inactive |
|
- *
NICE has avoided the term ‘dry early’ AMD which can easily be confused with ‘late dry’ AMD especially when the term ‘dry’ is used alone. NICE has also avoided using the term ‘advanced’ AMD since this may be confused with having advanced visual loss (which may occur in most if not all of the categories of AMD).
K.2. Neovascular AMD: Subtype descriptions and clinical features in the evidence
| Clinical Features | |||||
|---|---|---|---|---|---|
| Colour and red free photographs | FA early/late | OCT | FA/ICG and OCT | ||
| Geographic atrophy | Any sharply demarcated area of apparent disappearance of retinal pigment epithelium, larger than 175 µm, with visible choroidal vessels, and in the absence of neovascular AMD [MIC] | Grading based on features observed in the stereoscopic fundus photographs and fluorescein angiograms. According to the revised criteria, GA was defined as an area in which the RPE was absent, as evidenced by hyperfluorescence on late-stage fluorescein angiograms plus one additional feature indicative of RPE atrophy, specifically: visible choroidal vessels, sharp edges, or marked excavation on either CFP or FA. Atrophic drusen (i.e., degenerating drusen associated with RPE atrophy at its margins) were not considered GA unless the drusenoid material was completely encircled by a 360° rim of atrophy. This distinction was made to include regressing drusen located underneath a larger area of atrophy and exclude individual drusen or areas of confluent drusen that are associated with early atrophic changes. [Brader] | |||
| Wet (the presence of serous or haemorrhagic retinal PED, a subretinal neovascular membrane, a subretinal haemorrhage, a periretinal fibrous scar or a combinatio n of the above- [MIC] [AREDS4], [CARMS])) | Classic/type 2 | “greyish subretinal lesion occasionally with a surrounding ring of hyperpigmentati on” [Jung] | Early intense, well-demarcated hyperfluorescence with a characteristic lacy pattern. [Jung]
An area of choroidal hyperfluorescence with well demarcated boundaries that could be discerned in the early phase of the angiogram [Maguire] Late Intense leakage originating from the area of early hyperfluorescence [Jung] Progressive pooling of dye leakage in the overlying subsensory retinal space that usually obscures the boundaries of the CNV [Maguire] | The early lacy hyperfluorescen ce corresponds to a linear collection of subretinal hyperreflective material directly above the RPE line. The leakage corresponds to intraretinal oedema and or subretinal fluid. [Jung] | |
| Occult/type 1 | “RPE elevation with irregular height and shape; pigment mottling” [Jung] | Early
Stippled hyperfluorescence within 1 or 2 minutes (fibrovascular PED), or lack of early hyperfluorescent signal (late leakage of undetermined source) [Jung] An area of stippled hyperflourescence appeared within 5 minutes [Maguire] Late Mild to moderate staining and/or leakage corresponding to the RPE abnormalities [Jung] Occult lesions were either fibrovascular pigment epithelial detachments or late leakage of an undetermined source. [Olsen] Persistent staining or pooling of dye by 10 minutes {Maguire] Serous pigment epithelial detachment was considered present when there was a uniform, smooth elevation of the retinal pigment epithelium with sharply demarcated, fairly uniform, early Hyperfluorescence that persisted into the late phase of the angiogram. [Maguire] | The area of staining corresponds to an elevation of the RPE line with sub-RPE material of mixed reflectivity, often with overlying subretinal fluid. Intraretinal fluid is less common [Jung] | ||
| Mixed | Mixed features type 1 and 2 [Jung]
Mixed features type 1 and 3 [Jung] Mixed features type 2 and 3 [Jung] | Early
Mixed 1 and 2: well-demarcated hyperfluorescent lacy with or without surrounding area of stippled hyperfluorescence. Mixed 1 and 3: Stippled hyperfluorescence with or without hot spot Mixed 2 and 3: Well-demarcated hyperfluorescence. No contrast of the hot spot. Late Mixed 1 and 2: leakage and staining Mixed 1 and 3: staining or leakage, often with cystoid macular oedema Mixed 2 and 3: intense leakage, often with cystoid macular oedema [Jung] | Mixed 1 and 2: Type 1 and type 2 findings. The area of stippled hyperflourescen ce corresponds to the type 1 findings extending beyond the type 2 findings.
Mixed 1 and 3: Type 1 and type 3 findings. The area of angiographic staining corresponds to the type 1 lesion extending beyond the type 3 findings. Mixed 2 and 3: type 3 and type 2 findings. [Jung] | Predominantly classic (equal/greater than 50%) [Cohen] [Holtz] [Olsen] Minimally classic (less than 50%) [Cohen] [Holtz] [Olsen] | |
| RAP/type 3 | Early
Early but focal, leakage often seen in close proximity to retinal vessels. May have retinal anastomoses Late focal intense leakage, often with cystoid macular edema [Jung] | There is an intraretinal focal hyperreflective lesion in an area of localised outer retinal disruption. Often, there is a focal defect and variable degree of elevation of the underlying RPE. This intraretinal lesion corresponds to the early focal FA leakage and manifests surrounding intraretinal cystic changes. [Jung] | “Subretinal, intraretinal, or preretinal juxtafoveal haemorrhages; dilated retinal vessels; lipid exudates; and retinal-choroidal anastomosis.” [Coscas] | ||
| Polyp/PC V/IPC | “Elevated orange-red lesions, characteristic polypoidal lesions at the edge of a branching vascular network on angiography, and prominent anterior protusion of the retinal pigment epithelium line in OCT images.” [Coscas] (FA/ICG and OCT) | ||||
K.3. Classification systems: supporting evidence
| NICE recommended classification | Definitions | Supporting evidence: agreement scores (kappa and crude agreement) | Prognostic scores (hazard ratios and time adjusted odds ratios) for developing neovascular AMD |
|---|---|---|---|
| Normal eyes | Normal- no signs of ARM at all
or Small drusen (<63 µm) only [MIC] | ||
| Early |
| Two moderate quality studies found agreement to be 0.73 between graders for the AREDS 9-step severity scale,
One moderate quality study found agreement to be 0.88 between graders for the AREDS 4-step severity scale One moderate quality study found agreement to be 0.86 between graders, and 0.78 between grading centres for the Clinical Age-Related Maculopathy Staging system (CARMS). Two moderate quality studies found agreement to be 0.75-0.82 between graders for the Modified International Classification of ARM. One moderate quality study found agreement to be 0.66-0.86 between grading centres for Harmonized Three Continent AMD Consortium Severity Scale. | RQ2) 17 studies showed a positive correlation between neovascular AMD and:
RQ 6)
|
| |||
| |||
| Wet active and wet inactive | The presence of serous or haemorrhagic retinal PED, a subretinal neovascular membrane, a subretinal haemorrhage, a periretinal fibrous scar or a combination of the above- [MIC], [AREDS4], [CARMS] | Three very low quality studies found agreement to be between 0.37 - 0.64 between graders for the basic MPS/Gass classification.
One very low quality study found agreement to be 0.59 between graders for the MPS/GASS classification with pigment epithelial detachment (PED) as a subgroup of occult. One very low quality study found agreement to be 0.65 between two classification systems for multiple graders using the MPS/GASS classification with additional subgroup for retinal angiomatous proliferation (OCT) and the basic MPS/GASS classification (FA). One moderate quality study found agreement to be 0.75-1.00 between graders for the MPS/GASS classification with serous pigment epithelial detachment (PED) as an additional criteria. One low quality study found agreement with “final diagnosis” to be 79.4- 91.1% (AMD with type 1 CNV), 33.3- 66.6% (AMD with type 1+2 CNV), 60.0- 100% (AMD with type 2 CNV), 83.3% (chorioretinal anastomosis (RAP)) 66.6%, (PCV with type 1 or 2 CNV), 95.6% (PCV without type 1 or 2 CNV), 100% (Other) using fundus photographs, FA, ICG and OCT as investigations. In another cohort, the same low quality study found agreement with “final diagnosis” to be 95.8 - 97.9% (AMD with type 1 CNV), 68.4 - 89.5% (AMD with type 1+2 CNV), 60.0- 100% (AMD with type 2 CNV), 80.0- 100% (chorioretinal anastomosis (RAP)) 66.6-87.5% (PCV without type 1 or 2 CNV), 75-100% (Other) using fundus photographs, FA, ICG and OCT as investigations. | |
| Dry | NA | Two moderate quality studies found agreement to be 0.75-0.82 between graders for the Modified International Classification of ARM.
One low quality study found agreement to be 0.536 (0.03-1.04) between graders for a CAPT classification of geographic atrophy alone. |
- Age-related macular degeneration classification - Age-related macular degenerati...Age-related macular degeneration classification - Age-related macular degeneration
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