An Unusual Chorioretinal Manifestation of Relapsing Polychondritis Coexisting with Scleritis: A Case Report

Abstract

Purpose: To report an atypical chorioretinal manifestation concomitant with severe scleritis in a patient diagnosed with relapsing polychondritis. Methods: A 53-year-old male, with a six-month history of recurrent bilateral auricular cartilage inflammation was admitted to our hospital. Clinical and pathological examinations of the auricular cartilage led to the diagnosis of relapsing polychondritis. Results: Ophthalmological examination revealed a localized elevation in the superonasal quadrant of the left fundus, where the scleritis was more severe than in other regions. B-mode ultrasonography revealed choroidal thickening and subretinal lesions in this area, along with a white retinal lesion identified during the fundus examination. Further investigation using optical coherence tomography (OCT) unveiled subretinal and retinal lesions in the same region, with dispersed cells into the vitreous cavity in a fountain-like pattern. After treatment with systemic corticosteroids and cyclophosphamide, there was an improvement in scleritis, conjunctivitis, and retinal vasculitis; however, the white retinal lesion progressed to retinal atrophy. Concurrently, a decrease in the number of vitreous cells emanating from the area of fountain-like leakage was noted. Conclusions: Retinal manifestations of relapsing polychondritis are underrepresented in the literature. This report presents a unique case of relapsing polychondritis with a localized retinal manifestation observed on OCT imaging, thereby broadening our understanding of the potential localized retinal presentations associated with this disease.