Sympathetic ophthalmia is an autoimmune disease of the eye skin that occurs after eye injury. The characteristic uveitis can result in bilateral blindness. Treatment focuses on controlling inflammation with steroids.
What is sympathetic ophthalmia?
In autoimmune diseases, the immune system attacks the body’s own structures. In the context of most autoimmune diseases, evidence can be provided via so-called autoantibodies, which is considered to be diagnostic. Autoimmune diseases can basically attack any body tissue. Science understands sympathetic ophthalmia as an autoimmune disease of the eye skin tissue. For sore throat in English, please visit gradphysics.com.
This disease is a uveitis, which is characterized by inflammation of the middle skin of the eye, uvea. The uvea consists of the choroid (choroid), the iris and the radiating body (corpus ciliare). In individual cases, the vitreous body is also involved in the inflammatory processes of uveitis. Sympathetic ophthalmia presents bilaterally as diffuse granulomatous panuveitis and occurs after trauma or repeated ocular surgeries.
Ophthalmia was known to be a hereditary disease in the ranks of the Luxembourg nobles and caused John of Bohemia, for example, to lose sight in both eyes. Scientific sources indicate a prevalence of one to nine cases per million people for sympathetic ophthalmia. This makes the disease one of the rather rare diseases and has not yet been conclusively researched.
Around one in 1000 people with a penetrating eye injury will develop sympathetic ophthalmia. Age does not seem to play a role in this context. The etiology of sympathetic ophthalmia remains unclear. The causes of the characteristic inflammation lie in a cell-mediated immune process and an inflammatory autoimmune reaction against self-antigens that are released during the initial injury.
The exact nature of the self-antigens has so far been considered controversial. Various risk factors for the disease have been documented in the past. In addition to bacterial infections, the documented risk factors include specific HLA antigens such as DR4, B40 or A11. There are thus indications of an involvement of genetic factors, which could also explain the family accumulation among the aristocratic Luxembourgers.
In about half of all cases, the autoimmune disease is preceded by an injury. More rarely, it is a prehistoric contusion in the sense of a bruise. The highest risk is after injury to the ciliary body. However, inflammatory processes have also been reported after surgical interventions. There seems to be a higher risk after posterior segment surgery than after anterior segment surgery.
Symptoms, Ailments & Signs
Patients with sympathetic ophthalmia complain of pain in the area of the inflamed eye. In addition, they react with photophobia to the slightest exposure to light. They are no longer able to accommodate in the sense of functional near and far adjustment. In addition, there is often a subjective visual disturbance in the sense of a distorted perception of the environment, which can be accompanied by a slight to significant reduction in vision.
Granulating uveitis in the anterior segment is accompanied by changes in the posterior segment. Such changes can be, for example, moderate to severe inflammation in the area of the vitreous body, but also correspond to other inflammations. In addition, the changes are often associated with yellowish-white lesions in the area of the pigment epithelium or Dalen-Fuchs nodules.
The inflammation often leads to severe retinal detachment or water retention within the choroid, which results in loss of vision. Discomfort also occurs outside of the eyes. These accompanying symptoms are often headaches or hearing loss. More severe accompanying symptoms are meningitis in the sense of meningitis or pleocytosis of the cerebrospinal fluid, in which the number of cells in the cerebrospinal fluid increases.
Diagnosis & course of disease
The doctor makes the diagnosis of sympathetic ophthalmia on the basis of the history and the clinical symptoms. In addition to fluorescein and indocyanine green angiography, B-scan ultrasonography or optical coherence tomography can be used as imaging methods to confirm the suspected diagnosis. The doctor uses laboratory tests to rule out causal infections.
Endophthalmitis and other forms of uveitis can be differential diagnoses if there is a proven history of ocular trauma. If patients do not have a history of ocular trauma, other diseases associated with granular uveitis are more likely. In this respect, sarcoidosis or the Vogt-Koyanagi-Harada syndrome and the uveal effusion syndrome come into question. The prognosis is rather unfavorable for patients with sympathetic ophthalmia. Later, bilateral blindness can occur.
Sympathetic ophthalmia is a rare complication following an eye injury or eye surgery. Due to the exposure of the uvea after an eye perforation, the disease causes antibodies to form in the middle of the eye (uvea). Fatally, the antibodies also attack the uvea of the healthy eye, leading to chronic inflammatory processes in both eyes.
Therefore, if sympathetic ophthalmia is left untreated, it can lead to bilateral blindness. In addition to severe uveitis, complications such as papilledema and secondary glaucoma can also occur. Papilledema, also called congestion papilla, is characterized by a bulging of the optic nerve head. The papilledema leads to blurred vision and headaches.
Secondary glaucoma is in turn the result of an increase in intraocular pressure and, in addition to severe eye skin inflammation, can also lead to blindness. To reduce the risk of sympathetic ophthalmia spreading from the injured eye to the healthy eye, the severely injured eye is sometimes removed within the first two weeks after the accident. However, this should only be done when there is no chance of restoring vision to the affected eye.
In most cases, however, long-term treatment with immunosuppressive drugs is used to suppress the immune system’s activity in autoimmune reactions against the uvea, thereby averting ocular blindness. However, therapy with immunosuppressants such as corticosteroids is associated with side effects due to the weakening of the immune system.
When should you go to the doctor?
A doctor should be consulted immediately if the impairment of vision persists or worsens. Pain in the eye area, hypersensitivity to light influences and difficulty in seeing clearly require the support of a doctor. If visual problems occur even in poorer light conditions, this is considered unusual and must be investigated and treated. If the outlines of objects in the distance or in the immediate vicinity of the person affected can only be seen clearly with great effort, the complaints should be presented to a doctor.
It is considered worrying if people or objects can only be perceived out of focus. The general risk of accidents and injuries is increased in these situations. To avoid any side effects or life-threatening situations, a doctor should be consulted. Headaches, a feeling of pressure in the eye area and hearing loss are health impairments that should be diagnosed.
If there are unsteady gaits, abnormal behavior and changes in personality, these peculiarities should be understood as warning signals. In many cases, they indicate an existing disease. There is a need for action because the person affected is threatened with irreparable damage. In the case of an increased body temperature, a general risk of illness, anxiety, exhaustion or diffuse exhaustion, the symptoms should be discussed with a doctor.
Treatment & Therapy
In acute cases, sympathetic ophthalmia is to be countered by the immediate closure of all wounds. Patients receive the highest doses of steroids for at least three months. Depending on the individual case, the steroid dose is gradually reduced until all symptoms resolve. This is followed by maintenance therapy for six to twelve months.
If the characteristic inflammation cannot be controlled with steroids, immunomodulators such as cyclophosphamide or cyclosporine can be tried. Surgical treatments such as removal of the affected eye have been performed in the past with rather limited success. Nevertheless, a surgical intervention is still discussed in individual cases.
As a rule, when a decision is made for surgical intervention, there is blindness in the triggering eye. Doctors rarely remove seeing eyes because the prognosis of the affected eye can be significantly better in individual cases. Without any treatment, the visual prognosis remains extremely unfavorable.
In these cases, the autoimmune process usually spreads to the other eye and can thus cause bilateral blindness. Recurrences and complications are within the realm of possibility. Therefore, regular check-ups of the patients are one of the most important therapy steps in the long term.
So far, sympathetic ophthalmia can only be prevented by avoiding trauma to the eye. Since eye injuries and eye surgeries can only be prevented to a certain extent, no reliable preventive measures are currently available.
Sympathetic ophthalmia is an inherited eye disease in which inflammation spreads in the injured or operated eye. The reason for this process is the release of antibodies, and the autoimmune process creates the foci of inflammation. Ophthalmological therapies and consistent aftercare are necessary to protect the patient from complete blindness.
In sympathetic ophthalmia, the inflammation also affects the other eye over a period of a few weeks to several years. Aftercare is aimed at preserving the (still) existing vision. A complete loss of sight must be counteracted. There are two methods for this in ophthalmology: surgical removal of the inflamed eye or drug treatment if both eyes are affected.
During an operation, it is prevented from spreading to the vision-capable eye. After the surgical therapy, the well-known postoperative aftercare begins. It is continued by the ophthalmologist after discharge from the clinic. He monitors the success of the healing process and also advises those affected on how to deal with only one intact eye.
If the patient was given immunosuppressive drugs as part of medical treatment, the ophthalmologist will check tolerability and healing progress. Follow-up care depends on the condition after therapy. Even after the therapy has been completed, vision usually remains restricted. The person concerned receives professional advice from a specialist in the new situation.
You can do that yourself
Sympathetic ophthalmia requires medical treatment. Self-help measures that patients can carry out on their own do not exist. It is particularly important that you consult a doctor immediately if you injure your eyes. Otherwise there is a risk that the autoimmune process will spread from one eye to the other.
In order to protect yourself from blindness in both eyes, a close check-up by an ophthalmologist should also take place after treatment. In this way, recurrences can be recognized and treated in good time. In everyday life, those affected should minimize the risk of injury to the eyes. Depending on the environment, appropriate remedial measures may include wearing safety goggles to prevent trauma.
Furthermore, following medical treatment for the eye disease, patients must take the prescribed corticosteroids and strictly follow the doctor’s recommendation. The doctor may also prescribe other medications that must be taken regularly. Especially in the case of immunosuppressants, long-term administration must be ensured. Patients should therefore integrate taking medication into their daily routine.
As part of self-help, occasional eye relief is advisable. Too much screen work or staying in a very dusty environment can lead to new complaints.