11. Sarcoidosis


1. Mutton-fat KPs (large and small) and/or iris nodules at pupillary margin (Koeppe) or in stroma (Busacca)


2. Trabecular meshwork (TM) nodules and/or tent-shaped peripheral anterior synechiae (PAS)


3. Snowballs/string of pearls vitreous opacities


4. Multiple chorioretinal peripheral lesions (active and atrophic)


5. Nodular and/or segmental periphlebitis (± candlewax drippings) and/or macroaneurysm in an inflamed eye


6. Optic disc nodule(s)/granuloma(s) and/or solitary choroidal nodule


7. Bilaterality (assessed by clinical examination or investigational tests showing subclinical inflammation)




Retinal and Choroidal Involvements


Multiple chorioretinal lesions are regarded to be the sign of retinal or choroidal granuloma which appears to be yellowish-white lesions in its active phase. The lesions in the shallow layer of the retina look similar to the scars of laser photo coagulation in its atrophic phase (Fig. 11.1). Only a few chorioretinal lesions sometimes appear (Fig. 11.2a), while a number of lesions are sometimes observed in a close formation (Fig. 11.2b).

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Fig. 11.1

Chorioretinal lesions composed of fresh yellowish-white lesions and atrophic laser-scar like lesions in the peripheral retina


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Fig. 11.2

(a) A few yellowish-white chorioretinal lesions are seen in the inferior part of the retina. (b) A number of chorioretinal lesions seen in close formation in the nasal part of the retina


Retinal lesions also appear as yellowish subretinal exudative lesions (Fig. 11.3), and FA shows mottled hyperfluorescence in the corresponding area (Fig. 11.4). Although the other area of the retina looks normal, FA sometimes shows extensive hyperfluorescence in the retina or the retinal veins.

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Fig. 11.3

Subretinal yellowish exudative lesions in the nasal-inferior part of the retina


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Fig. 11.4

Fluorescein angiography (FA) shows mottled hyperfluorescence corresponding to the subretinal exudative lesions (a–d). Although the other area of the retina looks basically normal in the color fundus photo (Fig. 11.3), FA shows small patchy hyperfluorescence in the whole fundus and segmental hyperfluorescence in the retinal vein


The chorioretinal lesions can present in the deeper layer of the retina or in the choroid (Fig. 11.5a). While FA does not detect such lesions, ICGA can show the presence of the chorioretinal lesions in the deeper layers as multiple hypofluorescence dots (Fig. 11.5b, c).

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Fig. 11.5

(a) The chorioretinal lesions in the deeper layer of the retina or in the choroid are seen in the posterior pole. (b) Fluorescein angiography does not detect the chorioretinal lesions in the deep layer of the retina or in the choroid, but show some hyperfluorescein dots corresponding to the chorioretinal lesions in the shallow layer of the retina. Segmental hyperfluorescein are seen in the temporal-inferior retinal vein. (c) Indocyanine green angiography can show the presence of the chorioretinal lesions in the deeper layers as multiple hypofluorescence dots


Choroidal granuloma is a rare form of intraocular sign of sarcoidosis. It is observed as subretinal yellowish lesion with dull margin (Fig. 11.6a). Ultrasound imaging shows the elevated lesion (Fig. 11.6b), and OCT shows the thickened choroid and elevated RPE (Fig. 11.6c). OCT also detects the subretinal infiltrates, and this may be shown as hyperfluorescein area by FA (Fig. 11.6d). ICGA shows broadly distributed dark spots, and choroidal folds surrounding the granuloma (Fig. 11.6e). Optic disc nodule also is a rare form of intraocular sign of sarcoidosis (Fig. 11.7). The specificity of choroidal granuloma and optic disc nodule is high in patients with sarcoidosis when compared to other types of uveitis (Takase et al. 2010; Acharya et al. 2018). Choroidal granuloma eventually causes chorioretinal atrophy and retinal pigment epithelium degeneration (Figs. 11.8 and 11.9).

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Mar 22, 2020 | Posted by in OPHTHALMOLOGY | Comments Off on 11. Sarcoidosis

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