Fundus photographs show several cream-colored chorioretinal lesions involving the posterior pole of the right (A) and left (B) eyes with no involvement of the superotemporal retina in the left eye (C). One month later, numerous new lesions appeared within the macula in the right (D) and left (E) eyes and the peripheral retina including the superotemporal retina in the left eye (F). Following immunosuppressive therapy, numerous chorioretinal scars developed with no new lesion formation (G-I).
Axial fluid-attenuated inversion recovery magnetic resonance imaging in September 2006 showed multiple white matter lesions, including 1 lesion involving the left frontal lobe (arrow) (A) that resolved following prednisone and mycophenolate mofetil therapy (B).
Yeh S, Lew JC, Wong WT, Nussenblatt RB. Relentless Placoid Chorioretinitis Associated With Central Nervous System Lesions Treated With Mycophenolate Mofetil. Arch Ophthalmol. 2009;127(3):341-343. doi:10.1001/archophthalmol.2009.12
Relentless placoid chorioretinitis (RPC) is characterized by retinal lesions similar in clinical and angiographic appearance to acute posterior multifocal pigment placoid epitheliopathy (APMPPE) and serpiginous choroidopathy but differing in its widespread distribution, the numerous lesions typically seen, and the rapidly progressive clinical course.1,2 No consistent systemic manifestations have been described in prior reports. We describe a patient with RPC accompanied by central nervous system (CNS) lesions requiring immunosuppressive medication to achieve disease remission.
A 20-year-old man had a 3-week history of an enlarging central scotoma in the left eye, followed by similar symptoms in the right eye. He denied any viral prodrome but reported a history of migraines. Visual acuity at the initial visit was 20/400 OD and 20/50 OS. Ophthalmic examination revealed white placoid lesions within the posterior pole resembling APMPPE, with no evidence of vitritis (Figure 1A-C). Three weeks later, visual acuity deteriorated to 1′/200E OU with multiple new peripheral lesions in each eye (Figure 1D-F). Prednisone, 60 mg/d, was initiated at this time. Six weeks later, visual acuity was 20/800 OD and 6′/200E OS. Ophthalmic examination revealed progressive retinal disease with 2+ vitreous cells in each eye and multiple new oval peripheral lesions amidst widespread large regions of retinal pigment epithelial scarring and atrophy. The patient also reported headaches, which he ascribed to migraines.
Complete blood cell count, erythrocyte sedimentation rate, angiotensin-converting enzyme level, syphilis IgG level, rapid plasma reagin level, Lyme titers, purified protein derivative test results, chest radiography results, and urinalysis results were unremarkable. Magnetic resonance imaging showed multiple hyperintense lesions in the right temporal lobe, left frontal lobe, and left corpus callosum (Figure 2). A magnetic resonance angiogram and cerebral arteriogram were performed because of concern for cerebral vasculitis, but they failed to show any cerebrovascular abnormalities. A lumbar puncture showed reactive lymphocytosis.
Mycophenolate mofetil, 1000 mg twice daily, was started, and prednisone was maintained at a dosage of 20 mg/d. The prednisone was then tapered off over 2 months. Visual acuity improved to 20/160 OD and 20/100 OS, and no new lesions were seen. At the 18-month follow-up, the patient has remained free of disease recurrence. His visual acuity and ophthalmic examination results have remained stable (Figure 1G-I). Additionally, a follow-up magnetic resonance image showed complete regression of the CNS lesions (Figure 2).
Our patient's clinical course was consistent with features of RPC with progressive disease while he was receiving corticosteroid therapy. Furthermore, several subcortical white matter lesions were seen during the acute phase of his disease, which were possibly due to transient ischemia from small-vessel vasculitis or inflammatory lesions. The differential diagnosis of multiple placoid chorioretinal lesions includes APMPPE, serpiginous choroidopathy, multifocal choroiditis, lymphoma, metastases, sarcoidosis, tuberculosis, and syphilis. An extensive medical workup was unrevealing in our patient.
Because of lesion progression despite prednisone, mycophenolate mofetil was initiated and showed effectiveness for both the retinal and CNS findings. The natural history of RPC is unknown, and it is unclear whether disease resolution would have occurred without immunosuppressive therapy. A prior series of RPC reported a mean duration of disease activity of 9 months with continued appearance of new lesions for up to 24 months.1 In our patient, new lesions appeared for 3 months after his initial visit despite receiving prednisone. Remission was achieved following 2 months of mycophenolate mofetil treatment.
The precise mechanisms underlying the CNS and retinal findings are unclear, and no definite evidence of vasculitis was found. However, the temporal relationship between the CNS lesions and retinal lesions suggests a related inflammatory disease mechanism, possibly a small-vessel vasculitis or other focal inflammatory disease. Previously, APMPPE has been associated with cerebral vasculitis,3,4 and APMPPE and RPC could reflect differing ends of a disease spectrum. Our case report supports the consideration of immunosuppressive therapy for RPC and highlights the importance of neuroimaging in certain patients with RPC.
Correspondence: Dr Nussenblatt, Laboratory of Immunology, National Eye Institute, Bldg 10, 10D45, 10 Center Dr, Bethesda, MD 20892-1857 (firstname.lastname@example.org).
Financial Disclosure: None reported.
Funding/Support: This work was supported by the Intramural Research Program of the National Eye Institute, National Institutes of Health. Dr Yeh is supported by the Heed Ophthalmic Foundation.