Sympathetic Ophthalmia

Excerpt

Sympathetic ophthalmia is a rare pathologic process characterized by bilateral uveitis following injury to 1 eye either from surgery or penetrating ocular trauma. Though this is a rare process, the condition is serious and can cause blindness in the injured eye (ie, the inciting eye) and the contralateral eye (ie, the sympathizing eye). The onset of symptoms typically occurs within 1 year of injury but can occur up to 66 years after the initial injury. Sympathetic ophthalmia, often called sympathetic uveitis, is an uncommon and severe eye condition triggered by an immune system response. This inflammation impacts both eyes following trauma or surgery to 1 eye, especially when uveal tissue is involved.

The eye experiencing the initial trauma or surgery is called the "inciting" eye, while the other, affected eye is termed the "sympathizing" eye. The onset of symptoms can occur anytime from a few days to many years post-injury, but most cases appear within 1 year of the inciting event. Historically, younger individuals were more frequently affected due to accidental injuries. Still, recent reports suggest an increased occurrence in older adults, possibly linked to the higher rates of ocular surgeries in this demographic. Sympathetic ophthalmia, although rare, carries a significant risk of causing vision loss in both eyes. The exact pathogenesis of sympathetic ophthalmia is unclear. The immune-privileged status of the eye occurs after a penetrating injury and exposure of previously sequestered uveoretinal antigens to the systemic immune system. The resulting inflammation in the eye is devastating and causes permanent blindness. Therefore, early detection and intervention with immunosuppression are vital.

The clinical presentation can vary, with symptoms typically developing weeks to months after the initial injury. In some cases, the onset is delayed for years, leading to challenges in diagnosis. Common symptoms include decreased vision, eye redness, pain, and sensitivity to light. Upon examination, ophthalmologists may find signs such as inflammation in the vitreous and anterior chamber, choroidal lesions, and, in some cases, the characteristic Dalen-Fuchs nodules, accumulations of inflammatory cells beneath the retina. Sympathetic ophthalmia is primarily diagnosed through clinical findings and the history of trauma or surgery to the eye. Ancillary tests such as ocular imaging and laboratory investigations can support the diagnosis but are not definitive. The importance of early detection and treatment cannot be overstated, as timely management can significantly alter the disease course.

Treatment typically begins with high doses of corticosteroids to suppress the immune response and control inflammation. Due to the chronic nature of sympathetic ophthalmia, many patients require long-term immunosuppression to maintain control of the inflammation and preserve vision. This often necessitates the use of steroid-sparing agents like methotrexate, azathioprine, or biologic drugs. The risk factors for developing this condition include the nature of the trauma, the extent of uveal tissue exposure, and possibly genetic predispositions. Given the potential severity, prevention is a key aspect of management in ocular trauma and surgery, with meticulous surgical techniques to avoid uveal tissue exposure and prompt, aggressive treatment of any postoperative inflammation. Understanding sympathetic ophthalmia has evolved with advances in immunology and ocular imaging yet remains an active research area. Studies continue to seek better ways to predict who is at risk, how to prevent the development, and how to optimize long-term management to prevent recurrences and preserve vision. Sympathetic ophthalmia is an enigmatic and potentially devastating condition that encapsulates the intricate interplay between the ocular tissues and the immune system. The capacity to affect vision dramatically shows the importance of preventive strategies during ocular surgery, the necessity for vigilance in the face of ocular trauma, and the evolving landscape of immunomodulatory therapies in ophthalmology.