Abstract
Purpose :
APMPPE was originally described to affect the retinal pigment epithelium, but recent studies point its origin towards the inflammation of choriocapillaris and choroid. It is considered self-limiting with favorable visual prognosis but patients requiring immunosuppressive treatment have been reported. Herein, we present clinical features of a multi-institutional case series pooled with a systematic review and meta-analysis of published literature, that required corticosteroids with or without immunomodulatory therapy.
Methods :
24 eyes of 13 patients with APMPPE from four tertiary centers, which failed to resolve spontaneously, were included. Systematic literature search was performed using Medline, EMBASE, Cochrane and Google search engines. Additional 64 eyes of 39 patients from 10 studies were included.
Results :
In our case series, the median age was 23 years, 61.5% (n=8) were females and 84.6% (n=11) had bilateral disease. 83.3% (n=20) eyes had subretinal fluid (SRF), which worsened with observation in one patient but resolved with treatment in all eyes. In 3 eyes, steroid tapering was associated with clinical worsening which prompted for longer treatment course. All patients received steroids and 54% (n=7) received immunomodulatory therapy (mycophenolate, azathioprine, methotrexate, cyclophosphamide). These interventions led to visual recovery, resolution of SRF and healing of lesions in all cases. Pooled data from 52 patients (88 eyes) had a median age of 23 years, 48.1% (n=25) were females and 84.6% (n=44) had bilateral disease. Forest plot analysis used to calculate a weighted estimate showed that 44% (95% CI: 30-63) had SRF, 4% (1-12) had choroidal thickening, 3% (1-16) had vasculitis, 2% (0-12) had papillitis (Figure 1); and 3% (1-8) required immunomodulatory therapy in addition to steroids (Figure 2). Risk of bias analysis showed high risk of bias and GRADE certainty of evidence was low.
Conclusions :
Although it is thought that APMPPE is self-limited, this meta-analysis shows that treatment may be necessary in certain cases. OCT might be particularly helpful when the disease is foveal involving, to identify SRF (even when shallow), which could signify chronicity and the need for treatment. Provided rarity of this disease, prospective studies evaluating disease indicators requiring treatment remains challenging.
This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.