Relentless Placoid Chorioretinitis Associated With Central Nervous System Lesions Treated With Mycophenolate Mofetil

Proposing a Neurotropic Etiology for Acute Posterior Multifocal Placoid Pigment Epitheliopathy and Relentless Placoid Chorioretinitis

“…The reported case of 3 family members with the HLA-B7 and HLA-DR2, two of which developed optic neuritis, and one APMPPE within 4 months is also suggestive of an infective etiology (31). CNS manifestations associated with APMPPE (8) and RPC (32) and pleocytosis occurrence (8,32) (most commonly associated with CNS viral infection) (33) also suggest a potential CNS origin for APMPPE/ RPC in keeping with the von Szily model.…”

Proposing a Neurotropic Etiology for Acute Posterior Multifocal Placoid Pigment Epitheliopathy and Relentless Placoid Chorioretinitis

“…The reported case of 3 family members with the HLA-B7 and HLA-DR2, two of which developed optic neuritis, and one APMPPE within 4 months is also suggestive of an infective etiology (31). CNS manifestations associated with APMPPE (8) and RPC (32) and pleocytosis occurrence (8,32) (most commonly associated with CNS viral infection) (33) also suggest a potential CNS origin for APMPPE/ RPC in keeping with the von Szily model.…”

Relentless Placoid Chorioretinitis: A Differential Diagnosis and Management Approach in a Challenging Case

“…However, this case exhibits atypical features, such as bilateral involvement in different stages of the disease and dense vitreous inflammation, which could indicate a more aggressive variant of SC or an alternative diagnosis like RPC, which is regarded as a variant within the SC spectrum. The chronicity, the combination of bilateral placoid lesions in various stages of evolution, the relentless progressive course despite initial corticosteroid treatment, the presence of active intraocular inflammation with vitritis and optic disc edema, and the positive response to aggressive immunosuppressive therapy strongly favor RPC over the other conditions [ 7 , 8 ].…”

Relentless placoid chorioretinitis: A review of four cases in pediatric and young adult patients with a discussion of therapeutic strategies

“…Jones et al ( 1 ) presented two cases where cyclosporine (100–150 mg/day) was added in combination with prednisolone with successful tapering of prednisolone without any recurrence of lesions. Two additional studies have found the effective use of prednisolone and MMF 1,000–2,000 mg/day with no recurrence of lesions for 15–18 months: Yeh et al ( 8 ) reported that remission was achieved following 2 months of MMF treatment with successful tapering of oral prednisone (initial dose 20 mg/day), while Amer et al ( 19 ) reported a case where a 3-day course of IV methylprednisolone (500 mg/day) with a tapering dose of prednisolone (1 mg/kg/day) and addition of MMF (500 mg × 2/day) for 1 month lead to no recurrence of lesions 15 months after the first presentation.…”

Proposing a Neurotropic Etiology for Acute Posterior Multifocal Placoid Pigment Epitheliopathy and Relentless Placoid Chorioretinitis

“…The reported case of 3 family members with the HLA-B7 and HLA-DR2, two of which developed optic neuritis, and one APMPPE within 4 months is also suggestive of an infective etiology (31). CNS manifestations associated with APMPPE (8) and RPC (32) and pleocytosis occurrence (8,32) (most commonly associated with CNS viral infection) (33) also suggest a potential CNS origin for APMPPE/ RPC in keeping with the von Szily model.…”

Relentless Placoid Chorioretinitis: A Differential Diagnosis and Management Approach in a Challenging Case

“…However, this case exhibits atypical features, such as bilateral involvement in different stages of the disease and dense vitreous inflammation, which could indicate a more aggressive variant of SC or an alternative diagnosis like RPC, which is regarded as a variant within the SC spectrum. The chronicity, the combination of bilateral placoid lesions in various stages of evolution, the relentless progressive course despite initial corticosteroid treatment, the presence of active intraocular inflammation with vitritis and optic disc edema, and the positive response to aggressive immunosuppressive therapy strongly favor RPC over the other conditions [ 7 , 8 ].…”

Relentless placoid chorioretinitis: A review of four cases in pediatric and young adult patients with a discussion of therapeutic strategies

“…Jones et al ( 1 ) presented two cases where cyclosporine (100–150 mg/day) was added in combination with prednisolone with successful tapering of prednisolone without any recurrence of lesions. Two additional studies have found the effective use of prednisolone and MMF 1,000–2,000 mg/day with no recurrence of lesions for 15–18 months: Yeh et al ( 8 ) reported that remission was achieved following 2 months of MMF treatment with successful tapering of oral prednisone (initial dose 20 mg/day), while Amer et al ( 19 ) reported a case where a 3-day course of IV methylprednisolone (500 mg/day) with a tapering dose of prednisolone (1 mg/kg/day) and addition of MMF (500 mg × 2/day) for 1 month lead to no recurrence of lesions 15 months after the first presentation.…”

Proposing a Neurotropic Etiology for Acute Posterior Multifocal Placoid Pigment Epitheliopathy and Relentless Placoid Chorioretinitis

“…The reported case of 3 family members with the HLA-B7 and HLA-DR2, two of which developed optic neuritis, and one APMPPE within 4 months is also suggestive of an infective etiology (31). CNS manifestations associated with APMPPE (8) and RPC (32) and pleocytosis occurrence (8,32) (most commonly associated with CNS viral infection) (33) also suggest a potential CNS origin for APMPPE/ RPC in keeping with the von Szily model.…”

Relentless Placoid Chorioretinitis: A Differential Diagnosis and Management Approach in a Challenging Case

“…However, this case exhibits atypical features, such as bilateral involvement in different stages of the disease and dense vitreous inflammation, which could indicate a more aggressive variant of SC or an alternative diagnosis like RPC, which is regarded as a variant within the SC spectrum. The chronicity, the combination of bilateral placoid lesions in various stages of evolution, the relentless progressive course despite initial corticosteroid treatment, the presence of active intraocular inflammation with vitritis and optic disc edema, and the positive response to aggressive immunosuppressive therapy strongly favor RPC over the other conditions [ 7 , 8 ].…”

Relentless placoid chorioretinitis: A review of four cases in pediatric and young adult patients with a discussion of therapeutic strategies

“…Jones et al ( 1 ) presented two cases where cyclosporine (100–150 mg/day) was added in combination with prednisolone with successful tapering of prednisolone without any recurrence of lesions. Two additional studies have found the effective use of prednisolone and MMF 1,000–2,000 mg/day with no recurrence of lesions for 15–18 months: Yeh et al ( 8 ) reported that remission was achieved following 2 months of MMF treatment with successful tapering of oral prednisone (initial dose 20 mg/day), while Amer et al ( 19 ) reported a case where a 3-day course of IV methylprednisolone (500 mg/day) with a tapering dose of prednisolone (1 mg/kg/day) and addition of MMF (500 mg × 2/day) for 1 month lead to no recurrence of lesions 15 months after the first presentation.…”