Aggressive Retinal vasculitis and Progressive Outer Retinal Atrophy Associated with Sjögren's Syndrome: A Case Report.

PURPOSE: Aggressive retinal vasculitis (RV) and progressive outer retinal atrophy associated with Sjögren's syndrome (SS) are exceptionally rare. This case emphasizes the necessity for early recognition and monitoring of unexplained visual symptoms in patients with systemic autoimmune diseases. METHODS: This case reported a 36-year-old female presenting with a six-month history of progressive bilateral vision loss preceded by persistent photopsia. The patient was diagnosed with non-infectious RV and outer retinal atrophy associated with SS according to ophthalmic multimodal screening and thorough systemic examinations. Genomic screening, serum tissue-based assay (TBA), serum tumor markers and classical paraneoplastic antibodies testing were performed to rule out hereditary retinopathy, autoimmune encephalitis, cancer-associated retinopathy (CAR) and other paraneoplastic visual syndromes. RESULTS: A systemic anti-inflammatory and immunosuppressive therapy was initiated. However, it failed to arrest the relentless retinal atrophy. The patient experienced progressive visual field constriction, ultimately culminating in the irreversible disappearance of outer retinal structure despite systemic stabilization. CONCLUSION: Early cognition and standardized therapy of fundus lesions in SS patients need further study to avoid permanent visual loss. A more profound understanding of the relationship between SS and RV is needed to guide treatment.