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Sympathetic Ophthalmia

Disease Details

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Description: Sympathetic ophthalmia is a rare, bilateral, granulomatous uveitis that occurs after ocular trauma or surgery to one eye, potentially causing inflammation in the uninjured eye.
Type: Sympathetic ophthalmia is an autoimmune disease. It is not known to have a genetic form of transmission. Instead, it typically occurs after a penetrating injury or surgery to one eye, leading to inflammation in the uninjured eye.
Signs And Symptoms: Eye floaters and loss of accommodation are among the earliest symptoms. The disease may progress to severe inflammation of the uveal layer of the eye (uveitis) with pain and sensitivity of the eyes to light. The affected eye often remains relatively painless while the inflammatory disease spreads through the uvea, where characteristic focal infiltrates in the choroid named Dalén–Fuchs nodules can be seen. The retina, however, usually remains uninvolved, although perivascular cuffing of the retinal vessels with inflammatory cells may occur. Swelling of the optic disc (papilledema), secondary glaucoma, vitiligo, and poliosis of the eyelashes may accompany SO.
Prognosis: Sympathetic ophthalmia is a rare, bilateral, granulomatous uveitis that occurs after penetrating injury or surgery to one eye. The prognosis for this condition can vary but often requires prompt and aggressive treatment to control inflammation and prevent vision loss in both eyes.

1. Prognosis:
- Early diagnosis and appropriate treatment are crucial for a favorable outcome.
- With timely intervention, including the use of corticosteroids and immunosuppressive agents, many patients can achieve good control of the inflammation.
- Delayed or inadequate treatment can result in significant visual impairment or blindness in both eyes.

2. Nan: Not applicable (No additional or specific point of information referred to as "nan" relevant for the prognosis of sympathetic ophthalmia).
Onset: The onset of sympathetic ophthalmia typically occurs days to weeks after a penetrating ocular injury or surgery to one eye, leading to an inflammatory response in the uninjured eye.
Prevalence: Sympathetic ophthalmia is quite rare, with an estimated prevalence of 0.1 to 0.3 cases per 100,000 individuals.
Epidemiology: Sympathetic ophthalmia is rare, affecting 0.2% to 0.5% of non-surgical eye wounds, and less than 0.01% of surgical penetrating eye wounds. There are no gender or racial differences in incidence of SO.
Intractability: Sympathetic ophthalmia is often considered a challenging disease to manage but is not necessarily intractable. With modern medical interventions, including corticosteroids and immunosuppressive therapies, many patients can achieve significant improvement in symptoms and preservation of vision. Early diagnosis and aggressive treatment are crucial for optimal outcomes.
Disease Severity: Sympathetic ophthalmia is a rare but severe bilateral diffuse granulomatous panuveitis that occurs after trauma or surgery to one eye. The severity can be significant, leading to vision loss if not promptly diagnosed and treated. It is an ophthalmic emergency requiring immediate attention to prevent potentially severe repercussions for vision in both eyes.
Healthcare Professionals: Disease Ontology ID - DOID:12029
Pathophysiology: Sympathetic ophthalmia is currently thought to be an autoimmune inflammatory response toward ocular antigens, specifically a delayed hypersensitivity to melanin-containing structures from the outer segments of the photoreceptor layer of the retina. The immune system, which normally is not exposed to ocular proteins, is introduced to the contents of the eye following traumatic injury. Once exposed, it senses these antigens as foreign, and begins attacking them. The onset of this process can be from days to years after the inciting traumatic event.
Carrier Status: Sympathetic ophthalmia is an autoimmune inflammatory condition that affects both eyes following trauma or surgery to one eye. There is no carrier status associated with it, as it is not a genetic or hereditary disease.
Mechanism: Sympathetic ophthalmia is a rare, bilateral, granulomatous uveitis that occurs after a penetrating injury or surgery to one eye, leading to an autoimmune response affecting both eyes.

Mechanism:
The primary mechanism involves the release of uveal antigens following trauma or surgery to one eye. These antigens are normally sequestered from the immune system but become exposed due to the injury. Once released, they sensitize T cells, which then mount an autoimmune response that can affect the uninjured (sympathizing) eye.

Molecular Mechanisms:
1. **Antigen Presentation**: Uveal antigens are picked up by antigen-presenting cells (APCs) such as dendritic cells, which migrate to lymph nodes and present these antigens to T cells, initiating an adaptive immune response.
2. **T-cell Activation**: This process involves the activation of CD4+ T lymphocytes, particularly Th1 and Th17 cells, which play key roles in mediating the inflammatory response.
3. **Cytokine Production**: Activated T cells release pro-inflammatory cytokines like IFN-γ, IL-17, and TNF-α, which contribute to the inflammatory milieu.
4. **B Cell Activation and Autoantibody Production**: B cells may also play a role by producing autoantibodies against retinal or uveal antigens, further propagating the immune response.

The result is a bilateral inflammation characterized by granulomatous inflammation within the uvea, potentially leading to severe visual impairment if not promptly treated. Treatment typically requires immunosuppressive therapy to control the autoimmune response.
Treatment: Because SO is so rarely encountered following eye injury, even when the injured eye is retained, the first choice of treatment may not be enucleation or evisceration, especially if there is a chance that the injured eye may regain some function. Additionally, with current advanced surgical techniques, many eyes once considered nonviable now have a fair prognosis.
However, only if the injured eye has completely lost its vision and has no potential for any visual recovery, prevention of SO is done by enucleation of the injured eye preferably within the first 2 weeks of injury. Evisceration—the removal of the contents of the globe while leaving the sclera and extraocular muscles intact—is easier to perform, offers long-term orbital stability, and is more aesthetically pleasing, i.e., a greater measure of movement of the prosthesis and thus a more natural appearance. There is concern, however, that evisceration may lead to a higher incidence of SO compared to enucleation. Several retrospective studies involving over 3,000 eviscerations, however, have failed to identify a single case of SO.
Once SO is developed, immunosuppressive therapy is the mainstay of treatment. When initiated promptly following injury, it is effective in controlling the inflammation and improving the prognosis. Mild cases may be treated with local application of corticosteroids and pupillary dilators. More severe or progressive cases require high-dose systemic corticosteroids for months to years. Patients who become resistant to corticosteroids or develop side effects of long-term corticosteroid therapy (osteoporosis and pathologic fractures, mental status changes, etc.), may be candidates for therapy with chlorambucil, cyclophosphamide, or ciclosporin.
Compassionate Use Treatment: Compassionate use treatment and off-label or experimental treatments for sympathetic ophthalmia may include:

1. **Corticosteroids**: High-dose systemic corticosteroids are the primary treatment to control inflammation.

2. **Immunosuppressive Agents**: When corticosteroids are not effective or cause significant side effects, immunosuppressive drugs such as cyclosporine, methotrexate, azathioprine, or mycophenolate mofetil may be used off-label.

3. **Biologic Agents**: Some biologic drugs, like infliximab or adalimumab, have shown promise in experimental settings or off-label use. These TNF-alpha inhibitors can help control inflammation in severe cases not responding to conventional therapy.

4. **Intravitreal Injections**: Intravitreal corticosteroids or anti-VEGF injections might be used in some cases to directly target intraocular inflammation.

5. **Research Therapies**: Ongoing clinical trials may explore novel immunomodulatory therapies or targeted biologic treatments. Participation in such trials falls under experimental treatment options.

6. **Plasmapheresis or Intravenous Immunoglobulin (IVIG)**: In refractory cases, plasmapheresis or IVIG has been considered, though these treatments are typically more experimental and reserved for severe cases.

These treatments should only be pursued under strict medical supervision and often within the framework of clinical research or compassionate use protocols.
Lifestyle Recommendations: For sympathetic ophthalmia, the following lifestyle recommendations can help manage the condition alongside medical treatment:

1. **Regular Monitoring:** Keep regular appointments with your ophthalmologist to monitor eye health and adjust treatment as needed.

2. **Medication Adherence:** Follow the prescribed medication regimen, including corticosteroids and immunosuppressive drugs, to control inflammation and prevent complications.

3. **Eye Protection:** Wear protective eyewear during activities that could injure your eyes to prevent further trauma.

4. **Healthy Diet:** Maintain a balanced diet rich in vitamins and nutrients that support eye health, such as leafy greens, fish rich in omega-3 fatty acids, and antioxidant-rich foods.

5. **Stress Management:** Practice stress-reducing activities like yoga, meditation, or breathing exercises, as stress can potentially worsen inflammation.

6. **Smoking Cessation:** Avoid smoking, as it can exacerbate inflammation and negatively impact overall eye health.

7. **Avoid Eye Strain:** Limit activities that strain your eyes, such as prolonged screen time, and take regular breaks during such activities.

These lifestyle habits, in conjunction with medical care, can help manage sympathetic ophthalmia and improve quality of life.
Medication: Sympathetic ophthalmia is a rare, bilateral, granulomatous uveitis that occurs after trauma or surgery to one eye. It can potentially lead to severe vision loss if not treated promptly. The mainstay of treatment is the use of corticosteroids to reduce inflammation. Systemic corticosteroids, such as prednisone, are often prescribed initially. If the response to steroids is inadequate or if long-term treatment is needed to avoid the side effects of steroids, immunosuppressive agents such as cyclosporine, azathioprine, methotrexate, or mycophenolate mofetil may be used. In some cases, biologic agents like infliximab or adalimumab may also be employed. Timely and aggressive treatment is crucial to preserving vision in affected patients.
Repurposable Drugs: Sympathetic ophthalmia is a rare, bilateral, granulomatous uveitis that occurs after a penetrating injury to one eye, potentially leading to vision loss in both eyes. While specific treatments focus on corticosteroids and immunosuppressive agents, there is ongoing research into repurposable drugs. Methotrexate, azathioprine, and cyclosporine are among the immunosuppressive agents that have shown promise. If exploring repurposing options, it is recommended to consult updated clinical trials or medical research for the most recent information on effective treatments.
Metabolites: Sympathetic ophthalmia is a rare, bilateral, granulomatous uveitis that occurs after trauma or surgery to one eye, potentially affecting the other (sympathetic) eye. Currently, specific metabolites associated with the disease are not well-documented. Research focuses more on the autoimmunity and inflammatory responses involved. Nanotechnology applications in treatment are emerging but not yet established.
Nutraceuticals: Nutraceuticals generally play a supplementary role in managing overall health but are not a primary treatment for sympathetic ophthalmia, an inflammatory condition affecting both eyes following trauma to one eye. Standard treatment focuses on immunosuppressive therapy and corticosteroids. There is limited scientific evidence supporting the efficacy of nutraceuticals specifically for sympathetic ophthalmia. Always consult a healthcare provider before considering any nutraceuticals as part of the treatment plan.
Peptides: Sympathetic ophthalmia (SO) is a rare bilateral granulomatous uveitis that occurs after a penetrating injury or surgery to one eye, potentially causing inflammation in the uninjured eye.

Peptides: In the context of sympathetic ophthalmia, research into peptides has explored their use as diagnostic markers or therapeutic agents aiming to modulate the immune response involved in this autoimmune condition. Specific peptides may be under investigation for their roles in reducing inflammation or preventing immune cells from attacking the eye’s tissues.

Nan: Nanotechnology in sympathetic ophthalmia may involve the development of nanoparticle-based drug delivery systems. These systems can improve the targeting and efficacy of anti-inflammatory or immunosuppressive medications, potentially reducing side effects and enhancing treatment outcomes for patients with this condition.

This research aims to offer innovative approaches to managing and treating this complex autoimmune response in the eye.