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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.

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.

Background: Candida endophthalmitis is a severe complication of candidemia. Currently, the recommended treatment of fungal endophthalmitis is a combination of intravitreal and systemic antifungal drugs, and in some cases vitrectomy is also required. Intravitreal therapies that are commonly used are amphotericin B and voriconazole, although recently the use of intravitreal caspofungin has been described in a few case reports. However, clinical experience with intravitreal caspofungin is still limited., Case Presentation: We report a case of bilateral candida tropicalis endophthalmitis, initially managed with repeated 100 μg/0.1 ml caspofungin intravitreal injections and posteriorly treated with pars plana vitrectomy in both eyes., Conclusions: Intravitreal caspofungin could be a safe intravitreal alternative to habitual antimycotic drugs in cases with resistant candida endophthalmitis. Abbreviations: Intensive Care Unit (ICU); Best-Corrected Visual Acuity (BCVA).

Purpose: To report a case of Purpureocillium lilacinum endophthalmitis., Methods: The case of a fungal endophthalmitis caused by Purpureocillium lilacinum documented in an immunocompetent patient with no apparent trigger., Results: A 64-year-old male with a two-month history of panuveitis in his left eye was referred to our hospital. Initially misdiagnosed as sympathetic ophthalmia due to a previous surgery on his right eye 4 months before the onset of the left ocular picture, the patient received corticosteroid treatment, leading to a rapid deterioration of the left eye condition. An urgent exploratory vitrectomy was performed to identify the underlying cause, revealing endophthalmitis. Microbiological investigation yielded Purpureocillium lilacinum as the causative agent. Despite intensive treatment, including intravitreal antibiotics and antifungals, along with another surgical intervention, clinical evolution remained unfavourable, ultimately leading to the evisceration of the affected eye., Conclusions: Purpureocillium lilacinum poses a rare yet sever threat as a causative agent of fungal endophthalmitis. Managing such cases is challenging due to the delayed identification, fungus's resistance to common antifungals, and its association with prior corticosteroid misuse in most patients. This case underscores the crucial importance of heightened clinical suspicion, early diagnosis, and the exploration of alternative treatment strategies in addressing Purpureocillium lilacinum endophthalmitis. The challenges posed by this rare fungal pathogen emphasize the need for a multidisciplinary approach and continued research to improve outcomes in these complex cases., (© 2024. The Author(s).)

Background: Myotonic dystrophy is an inherited disease characterized by progressive muscle weakness and myotonia. It is a multisystemic disorder that affects different parts of the body, including the eye. Dysfunction of ocular muscles, ptosis and cataract are the most common ophthalmologic manifestations, but it can also present with pigmentary changes in the retina. This report presents and discusses an unusual case of a pigmented pattern dystrophy simulating a fundus flavimaculatus in a patient with myotonic dystrophy., Case Presentation: We present a case of a woman with a history of myotonic dystrophy and complaints of progressive vision loss who presented bilateral retinal pigmentary changes in posterior pole and midperiphery. The characteristics and distribution of pigmented deposits, as well as ancillary tests, showed a retinal phenotype compatible with a multifocal pattern dystrophy or a fundus flavimaculatus., Conclusions: There are a few publications about retinal disorders in patients with myotonic dystrophy. When macular area is affected it tends to adopt a patterned-shape defined as butterfly dystrophy or reticular dystrophy. To our knowledge, this is the first report of a patient with myotonic dystrophy and multifocal pattern dystrophy or fundus flavimaculatus.