Posterior Scleritis Associated with Late-Onset Takayasu Arteritis: A Case Report.

PURPOSE: To describe a rare case of posterior scleritis associated with late-onset Takayasu arteritis. METHODS: A case report of a 75-year-old female presenting with posterior scleritis, eventually diagnosed with late-onset Takayasu arteritis was described. Several medical evaluation visits were conducted, including ophthalmological and rheumatological follow-up. Multiple diagnostic tests were carried out, and the cornerstones of the treatment were corticosteroids and the IL-6 inhibitor tocilizumab. RESULTS: The patient was admitted to our hospital's emergency room with right unilateral eyelid edema, chemosis and red eye. She had previously experienced two episodes that were misdiagnosed as preseptal cellulitis. The presence of choroidal folds and the T sign on the ultrasound exam were highly suggestive of posterior scleritis. Later, the patient was diagnosed with Takayasu arteritis, a type of large vessel vasculitis. Treatment with steroids was started, which was later switched to IL-6 inhibitors to achieve better control of the systemic disease. CONCLUSION: Posterior scleritis is often misdiagnosed, necessitating high clinical suspicion. Multimodal diagnosis is important to establish an accurate diagnosis. Up to 34% of cases may be associated with a systemic disease. This is the first case described in the literature of posterior scleritis associated with late-onset Takayasu arteritis.

Familial Mediterranean Fever-Associated Retinal Vasculitis: A Rare Manifestation Successfully Managed with IL-1 Pathway Inhibitors.

PURPOSE: To investigate the rare manifestation of retinal vasculitis in Familial Mediterranean fever (FMF) and its correlation with specific gene mutations, particularly the MEFV gene, with a focus on the severity of phenotypes and systemic vasculitis. METHODS: A case report of a 45-year-old Armenian patient with FMF history and dual mutations (M680I and M694V) was analyzed. Clinical assessments, including ocular examinations, were conducted at various stages of the disease. Treatment modalities, including prednisone, Anakinra, and Canakinumab, were administered and their effectiveness was assessed. RESULTS: The patient presented with bilateral ocular pain and decreased vision, exhibiting acute anterior uveitis, perivascular hemorrhages resembling Roth spots, and subsequent features of persistent vascular sheathing and cotton-wool spots. Dual mutations, especially M694V, were associated with a severe phenotype and systemic vasculitis. Treatment with prednisone induced remission, and IL-1 pathway inhibition with Anakinra and Canakinumab successfully managed relapses. CONCLUSION: This case underscores the rarity of retinal vasculitis in FMF, particularly involving arteries, and highlights the correlation between specific gene mutations (M680I, M694V) and disease severity. The successful management with IL-1 pathway inhibitors suggests a potential therapeutic approach. Increased clinical awareness, further research, and reporting are crucial for optimizing the understanding and treatment of FMF-related ocular manifestations.