Name

Choroidite ampigineuse en tant que manifestation initiale d'une sarcoïdose systémique

Relentless placoid chorioretinitis (RPC) is a rare, bilateral disease of the retinal pigment epithelium. RPC may be associated with autoimmune or infectious diseases, although the exact cause is often unknown. We report an undiagnosed case of systemic sarcoidosis first presenting with bilateral, relentless placoid chorioretinitis (RPC).

A 29-year-old Caucasian man presented with bilateral visual loss. Slit lamp examination showed bilateral anterior uveitis und vitritis. Fundus examination showed  yellow active placoid lesions in the posterior pole and grey placoid lesions (of different stages) in the midperiphery of both eyes. Fundus Autofluorescence (FAF)  demonstrated hyper-autofluorescence lesions in the macula,  hypo-autofluorescence  lesions in the mid-periphery of the retina. Fluorescein Angiography (FA) showed early hypofluorescence and late hyperfluorescence of the active placoid lesions in both eyes.

SD-OCT demonstrated disrupted foveal contour, focal subfoveal fluid and hyperreflective lesions in the outer retinal layers in both eyes

Based on the fundus and multimodal imaging findings, as well as the atypical location of multiple lesions at different stages extending from the posterior pole to the mid-peripheral retina, we diagnosed our patient with relentless placoid chorioretinitis. Further evaluation revealed kidney dysfunction at KDIGO stage G3B. A renal biopsy was then performed, which showed focal transmural epithelioid cell arteritis, severe epithelioid cell non-necrotizing granulomatous interstitial nephritis and diffuse moderate acute tubular injury. High-dose corticosteroids were started for sarcoidosis with ocular and renal involvement. At follow-up 1 month after initial presentation, visual acuity and inflammatory signs improved.. Systemic prednisone was slowly tapered.  There was no evidence of disease recurrence or progression on funduscopy or FAF  seen after discontinuation of oral prednisone.

This case illustrates a presentation of systemic sarcoidosis initially presented as RPC. To our knowledge, this is the first reported case of sarcoidosis with RPC, highlighting the value of ophthalmological findings in detecting systemic diseases like sarcoidosis.