Posterior scleritis with secondary uveal effusion unmasking peripapillary pachychoroid syndrome

Purpose To describe a case of posterior scleritis with secondary uveal effusion in which treatment of the underlying inflammatory process unmasked a coexisting peripapillary pachychoroid syndrome (PPS). Methods Report of a 71-year-old patient presenting with decreased visual acuity (20/100) and periocular pain in the right eye (OD). Examination included best-corrected visual acuity (BCVA), slit-lamp and dilated fundus examination, spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and contrast-enhanced orbital magnetic resonance imaging (MRI). Laboratory testing was performed. High-dose intravenous methylprednisolone was followed by an oral taper, as well as systemic immunosuppression with subcutaneous methotrexate. Results At presentation, OD showed disc edema, macular serous detachment, and four-quadrant choroidal detachments; the left eye (OS) was asymptomatic with the exception of choroidal folds. MRI demonstrated posterior scleral enhancement and confirmed choroidal detachments. FA showed mottled hyperfluorescence without vasculitis; ICGA showed no choriocapillaritis or stromal granulomas. Following intravenous corticosteroids there was symptomatic improvement, BCVA increased to 20/32, and choroidal detachments resolved. Three weeks later, despite resolution of subretinal fluid, bilateral peripapillary intraretinal fluid/schisis developed. Considering short, hyperopic ocular anatomy, choroidal folds, peripapillary thickening, and steroid exposure, PPS within the pachychoroid spectrum was considered. Gradual corticosteroid de-escalation led to bilateral fluid resolution and final BCVA of 20/25 OD 20/20 OS. Conclusion Posterior scleritis may coexist with PPS, and treatment with corticosteroids may worsen pachychoroid syndrome. Multimodal imaging plays a critical role in distinguishing posterior scleritis from granulomatous choroiditis and is essential in leading to a prompt diagnosis and treatment.