Sympathetic Ophthalmia – a Contribution to Immunology, Clinic and Current Imaging

Abstract

Abstract Background Sympathetic ophthalmia (SO) is a rare inflammation of an operated or injured eye that spreads to the fellow eye. It is typically a bilateral granulomatous panuveitis. The traumatized eye is referred to as inciting eye and the fellow eye as sympathizing eye. The pathophysiology of the disease is not entirely understood, but there is strong evidence of an autoimmune genesis. Patients/Material and Methods A selective literature search on epidemiology, immunology, clinical features and risk factors of SO was carried out. In addition, our own experience using multimodal imaging for this clinical entity was introduced. Results In the literature, the incidence after traumatic eye injuries is 0.1 – 3% and approximately 0.01% after intraocular surgery. Among the iatrogenic causes, vitreoretinal surgery has the highest rate of SO, presumably due to disruption of the blood-retinal barrier and involvement of retinal and choroidal tissue, which are susceptible to anterior traction, phthisis and chronic inflammation. In 90% of patients, the disease develops within a year following the eliciting event and is associated with a potentially bilateral risk of blindness. Typical symptoms include bilateral visual impairment with photophobia, dull pain and photopsia. The spectrum of clinical manifestations ranges from granulomatous anterior uveitis and vitritis, to choroiditis, serous retinal detachment and Dalen-Fuchs nodules in the context of posterior involvement. The diagnosis of SO is generally based on clinical presentation and is supported by imaging methods. These primarily comprise fluorescein and indocyanine green angiography, which are increasingly being supplemented by non-invasive methods such as optical coherence tomography. They can provide important information for assessment of severity, differential diagnosis as well as for disease monitoring. The differential diagnosis includes i. a. Vogt-Koyanagi-Harada syndrome, ocular sarcoidosis and the rare phacoanaphylactic endophthalmitis. Immediate systemic high-dose steroid therapy is used as initial treatment. The course of the disease is often relapsing to chronic progressive. Immunomodulators such as ciclosporine A, azathioprine, cyclophosphamide, mycophenolate mofetil, and biologics are increasingly being used and contribute to the significantly better prognosis of the disease. Generally, SO can be triggered by any kind of intraocular intervention. Conclusion SO remains a threatening clinical diagnosis that poses diagnostic and therapeutic challenges. It can be triggered post-traumatic, but also any intraocular surgery. This should be taken into account when assessing the indication for intraocular eye surgery, especially in eyes with reduced visual outcome.