Core Messages

• The common denominator in primary inflammatory choriocapillaropathies is chori-ocapillaris non-perfusion and secondary ischaemia of the outer retina. Indocyanine green angiograhy and early fluorescein angiographic frames show choriocapillaris non-perfusion. Electroretinography shows outer retinal dysfunction and late fluorescein angiographic hyperfluorescent frames are explained by the ischaemia of the outer retina causing retinal capillaries to leak

• The clinical differences between the primary inflammatory capillaropathies (PICCP) could possibly be explained by the level and the severity of the inflammatory insult to the choriocapillaris circulation

• Angiographic signs in inflammatory choriocapillaropathies: ICGA 1: in the acute phase of disease, patchy or geographical ICGA hypofluorescent areas of variable sizes present in the early, intermediate and late angiographic frames but are usually more clearly visible on the late frames after partial wash-out of ICG from choroid; ICGA 2: in the post-acute phase, ICGA hypofluorescent areas represent choroidal atrophy and scarring; ICGA 3: there are hyperfluorescent ICGA rims around progressing (serpiginous) lesions; FA 1: in the acute phase of disease, early FA hypofluorescent areas indicate choriocapillaris non-perfusion; FA 2: in the acute phase of the disease, late FA hyperfluorescent areas go from faint to profuse depending on the severity of ischaemic process at the level of the outer retina;

FA 3: in the postacute phase, there are zones of alternating areas of window effects and masking effects indicating chorioretinal atrophy and scars; in the convalescent phase of disease, regression of FA signs lags behind normalization of ICGA signs

• Multiple evanescent white dot syndrome (MEWDS) and acute idiopathic blind spot enlargement (AIBSE): (1) viral flu-like syndrome often found in preceding fortnight in up to 60% of cases; (2) usually unilateral and unique episode; (3) symptoms: photopsias, scotomata, visual loss that can be severe in some cases; (4) visual loss found in >90% of cases: drop of visual acuity from slight to severe, visual field changes and blind spot enlargement; (5) fundus findings: discrete discolorations in midperiphery and granular aspect of macula; (6) ERG abnormal in 80% of cases; (7) ICGA: hypofluorescent dots and peripapillary hypofluorescence in acute phase resolving in 4-8 weeks; (8) FA: hypo-fluorescence (early), discretely hyperfluorescent foci (late) or absent FA findings ± cystoid macular edema; disc hyperfluores-cence;(9) spontaneous resolution of signs and symptoms within 6-10 weeks in ± all cases; (10) AIBSE:entity described before the ICGA era behaving as MEWDS without fundus signs that have probably resolved or are subclinical

• APMPPE/AMIC: (1) viral flu-like syndrome often found in patient history; (2) symptoms: visual loss, scotomata, photopsias; (3) yellowish deep bilateral discoloration at posterior pole (with serous exudative retinal detachments in hyperacute cases);

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