Progressive subretinal fibrosis and uveitis syndrome: clinical course and multimodal imaging analysis in a late-onset case
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Published on: December 17, 2021
Alessandro Feo1,2, Phillip P Le3, Kirk Hou4,3
1Stein Eye Institute, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA. alessandrofeo.96@gmail.com.
Purpose:
To describe the longitudinal clinical and multimodal imaging findings of a patient with progressive subretinal fibrosis and uveitis (SFU) syndrome.
Case Presentation:
Case report. An 85-year-old woman with a history of cardiomyopathy, systemic hypertension, hyperlipidemia and hypothyroidism presented with a 5-day history of acute bilateral visual decline. At baseline, best-corrected Snellen visual acuity was 20/80 OD and 20/400 OS. Fundus examination revealed multifocal chorioretinal lesions with fibrotic subretinal changes and vitreous inflammation. Fundus autofluorescence demonstrated scattered areas of hypoautofluorescence with hyperautofluorescent borders, and fluorescein angiography revealed window defects with late staining but no leakage. Spectral-domain optical coherence tomography (SD-OCT) showed diffuse retinal pigment epithelium (RPE)-Bruch's membrane (BrM) splitting and overlying fuzzy subretinal hyperreflective material (SHRM). Primary vitreoretinal lymphoma and multifocal choroiditis were initially suspected; however, systemic and uveitis work-up was negative. The patient's advanced age was atypical for new-onset SFU and further contributed to diagnostic uncertainty. Despite oral corticosteroid treatment and intravitreal dexamethasone implants OU, the disease progressed relentlessly, leading to widespread subretinal fibrosis and chorioretinal atrophy, and severe visual decline (20/100 OD, counting fingers OS at 12 months).
Conclusion:
SFU is a rare, aggressive, and underrecognized immune-mediated uveitic syndrome that can masquerade as neoplastic or inflammatory chorioretinopathies. This atypical late-onset case expands the known age spectrum of the disease and highlights the importance of considering SFU in rapidly progressive chorioretinal fibrosis in older adults. OCT biomarkers - particularly RPE-BrM splitting and fuzzy SHRM - may signal early SFU, emphasizing timely recognition and immunomodulatory therapy to prevent vision loss.