Cyclosporine and prednisolone combination therapy as a potential therapeutic strategy for relentless placoid chorioretinitis

Abstract

Purpose: Relentless placoid chorioretinitis (RPC) is a new disease concept that was proposed by Jones et al. in 2000. Some cases of RPC have been reported; however, a treatment strategy has not yet been established. We report herein four cases of patients diagnosed with RPC.

Observations: We experienced four cases of RPC in patients aged 24-51 years. All patients exhibited retinal lesions similar to that seen in acute posterior multifocal placoid pigment epitheliopathy or serpiginous choroiditis from the posterior pole to the surrounding region. Although patients underwent systemic prednisolone (PSL) therapy, recurrence was observed and the retinal scar formation was progressive; they were then diagnosed with RPC. In all cases, cyclosporine (CyA) was administered in addition to PSL, no recurrence was observed thereafter.

Conclusions and importance: RPC is a rare disease, and a treatment strategy has not yet been established. CyA and PSL combination therapy is considered to be effective in the treatment of RPC.

Keywords: APMPPE; Cyclosporine; Prednisolone; Relentless placoid chorioretinitis; Serpiginous choroiditis.

Fig. 1

Fundus of Case 1 OD. At the first visit numerous placoid lesions mixed with fresh lesions (white arrowhead) and old scar lesions were scattered from the posterior pole to the peripheral retina (a). In fluorescein angiography these fresh lesions were the hypofluorescence in the early phase (116 seconds) (b) and the hyperfluorescence in the late phase (406 seconds) (c) (white arrowhead). In contrast, scar lesions were the hypofluorescence surrounded by hyperfluorescence with no late leakage.

Fig. 2

Fundus of Case 1 OD after steroid pulse therapy. Fresh lesions were resolved with steroid pulse therapy, and some of them changed to scar lesions (a). However, two months from the initial visit, recurrence was seen OD. New white retinal lesions appeared in the temporal macula OD (b).

Fig. 3

Fundus of Case 2 at the first visit. Multiple white placoid fresh lesions and scar lesions were seen OU at the first visit. They changed to scar lesions after the corticosteroid therapy.

Fig. 4

Fundus of Case 3 at the first visit. Numerous scattered scar lesions accompanied with fresh white lesions (white arrowhead) were seen at the initial visit OS (a). (Numerous scattered scar lesions accompanied with fresh white lesions were also seen OD, however, fundus photograph OD could not be taken due to the posterior iris synechia.) Two month later, new white chorioretinal lesions (white arrowhead) appeared in addition to old scar lesions OS (b).

Fig. 5

Slit-lamp image of Case 4 at the first visit. Hypopyon along with fibrin formation was seen in the anterior chamber OU (photo shows right eye).

Fig. 6

Fundus of Case 4 after several recurrences. Numerous orange color chorioretinal scars were widely spread from the posterior pole to peripheral retina OU. Right eye (a), Left eye (b).