The Eales syndrome is a vasculitis in the eye, which is accompanied by damage to the retina, bleeding of the vitreous and Visusverlusten. The causes of the disease have not yet been clarified and can correspond to the most varied of contexts, from autoimmunological processes to tuberculosis symptoms. Symptomatic treatment of the syndrome includes drug and ophthalmic surgical steps.
What is Eales Syndrome?
In a so-called vasculitis is an inflammatory disease that affects the blood vessels. Vasculitides can occur in almost any tissue and have different causes. Eales syndrome belongs to the group of vasculitides and corresponds to an eye disease that results in retinal damage with loss of vision and vitreous hemorrhage.
According to usvsukenglish, the disease is named after its first writer Henry Eales. The English ophthalmologist described the phenomenon as early as the end of the 19th century. In the medical literature, the disease is also known as angiopathia retinae juvenilis or periphlebitis retinae.
Sometimes the phenomenon is also referred to as idiopathic perivasculitis of the retina or idiopathic retinal pervasculitis. The prevalence for the eye disease is so far unknown. Male adults are predominantly affected by Eales syndrome. The average age of onset is between the ages of 20 and 30.
The causes of Eales syndrome are just as unclear as the genesis of the disease. Since no familial accumulation was observed in the cases documented so far, it is probably not a hereditary disease. Common backgrounds could not be identified on the basis of the previous cases. Therefore the disease remains idiopathic.
The assignment to the group of forms of vasculitides at least explains the reasons for the loss of vision in the context of the syndrome. Inflammation of the supplying vessels is the initial stage of the syndrome. This inflammatory stage is associated with periphlebitis of the peripheral areas of the retina. In the ischemic stage, sclerosis of the retinal veins occurs, which in the subsequent stage of proliferation turns into new blood vessels with bleeding.
These processes lead to retinal detachment. According to scientific assumptions, there is a multifactorial genesis of autoimmunity, Mycobacterium tuberculosis, oxidative stress and possibly coagulopathy for the syndrome. As a rule, the disease occurs spontaneously and without any inflammatory or traumatic events.
Symptoms, ailments and signs
At the onset of the disease, patients with Eales syndrome often show no manifest symptoms. Early symptoms of the disease include, for example, blurred vision, which leads to painless loss of vision. The visual acuity is only in the rarest of cases worse than 20/200.
Various complaints point to vascular damage: Rubeosis iridis occurs in the anterior chamber of the eye and, in individual cases, glaucoma caused by neovascularization. The posterior chamber of the eye shows vitreous hemorrhage and traction amotio.
In later stages, proliferative retinopathy with retinal bleeding often occurs. Retinal periphlebitis is also one of the most common symptoms. Symptoms of the central nervous system have been described as complications in isolated cases. These symptoms include primarily sensory disorders, speech disorders and loss of movement.
Diagnosis & course
The diagnosis of Eales syndrome takes place under the exclusion of differential diagnosis of diseases such as sarcoidosis, the closure of retinal vein branches and other proliferative diseases of the retina. The diagnostic agent of choice is usually fluorescein angiography of the fundus.
In addition to this diagnostic test, a sonography can be performed, which provides helpful information, especially in the case of retinal detachment and vitreous hemorrhage. Various authors also recommend an analysis of blood coagulation. Basically, the prognosis for patients with Eales syndrome is rather favorable. Complete blindness was only documented in isolated cases.
Nonetheless, the course varies from person to person. Complete regression is conceivable, but progression to retinal detachment is just as conceivable. Recurring complications have occurred in individual cases.
When should you go to the doctor?
Since Eales syndrome can, in the worst case, lead to complete blindness, an examination by a doctor should definitely be carried out. Self-healing does not occur with this disease. The doctor should usually be seen if the person concerned has blurred vision or if there is a general deterioration in vision. This deterioration usually occurs very suddenly.
Speech disorders or paralysis also indicate Eales syndrome and must definitely be examined. Eales syndrome can also lead to restrictions in movement, which must also be examined by a doctor. First and foremost, an ophthalmologist can be consulted with this disease. The earlier the disease is diagnosed, the higher the likelihood of a positive course of the disease. Since the symptoms of the disease can reappear even after successful treatment, the person affected should also take part in regular examinations after treatment.
Treatment & Therapy
A causal therapy is currently not available for patients with Eales syndrome. The reason for this lies in the insufficient clarification of causal factors. Causal therapies address the primary cause of a disease in order to stop the disease process at the root.
By eliminating the cause, the individual symptoms are also permanently resolved with causal therapy steps. Although the idiopathic Eales syndrome does not offer a causal starting point, at least symptomatic treatment approaches are available. Symptomatic treatment steps are based on the symptoms in the individual case and in Eales syndrome are mainly stage-dependent.
For example, in the perivasculitis stage, patients are given corticosteroids. This conservative drug treatment can correspond to a systemic or peri-ocular form of therapy. Systemic gift means the effect that encompasses the body. In this context, the corticosteroids are administered orally or via an infusion and thus act on the entire organism via the bloodstream.
Peri-ocular administration at the location of the pathological event is gentler and more targeted. Since speculation suggests that autoimmunological processes are probably involved in the inflammatory disease, immunosuppressants such as azathioprine or cyclosporine can be given on a trial basis. The immune system of the patient is dampened by these means.
Because of the tuberculosis factors that may be involved, some scientists recommend an additional tuberculostatic therapy. However, this approach is considered controversial. In the proliferative stage, photocoagulation is the treatment option of choice. Vitreous retinal surgery can also be used, especially in the case of prolonged vitreous bleeding or impending adhesions with the risk of retinal detachment.
Outlook & forecast
The course of the Eales syndrome is very different. Both regression and progression of the disease can occur. So far there is no causal therapy. It can only be treated symptomatically. Often there is a loss of vision. Nevertheless, complete blindness is very rare, even in untreated people.
Problems arise when the vitreous hemorrhage and retinal hemorrhage occur over a long period of time. Also retinal detachments occur. If the vitreous hemorrhage lasts longer than three months or there is a risk of retinal detachment, a vitreous retinal operation is necessary. The constant formation of new blood vessels causes growths which, in addition to detachment of the retina, can also lead to glaucoma. Without treatment, there is a risk of loss of sight.
All treatment methods can alleviate the most severe symptoms, but have no effect on the course of the disease. Since the causes of an ealess syndrome are not yet fully understood, no causal therapy has yet been found. As already mentioned, the symptoms can regress completely or progress further, both with and without treatment.
As the disease progresses, vascular breakdown and new vascular formation alternate with one another. During the formation of new vessels (proliferation), new cells are constantly being formed, which lead to overgrowth. If there is a risk of retinal detachment caused by proliferation, photocoagulation is recommended as a therapy in order to completely reattach the retina.
So far, Eales syndrome cannot be prevented, as the causes are still considered idiopathic. Only when the causal factors involved have been finally clarified are preventive starting points available.
The measures or options for follow-up care are very limited in Eales syndrome. In any case, the affected person is dependent on a quick diagnosis with subsequent treatment, since otherwise untreated a complete loss of vision can occur. This can be irreversible and can therefore no longer be treated. Therefore, early detection of the syndrome is in the foreground in Eales syndrome.
If the first signs or symptoms of this disease appear, an ophthalmologist must be contacted immediately. In most cases, patients with Eales syndrome rely on medication to relieve the symptoms. Care should be taken to ensure that it is taken regularly with the correct dosage.
If anything is unclear or if you have any questions, a doctor should always be contacted first so that incorrect assumptions are not made. In children, parents should check the correct intake. Regular checkups by an ophthalmologist are also very important in order to check the stage of Eales syndrome. In some cases, surgical interventions may also be necessary. After such an operation, the eye should be particularly well protected, and a bandage is usually worn around the eye.
You can do that yourself
Eales syndrome is a relatively rare eye disease, the causes of which are still largely unknown. Everyday life with this disease is very different, depending on how severe the symptoms are. The bursting of blood vessels in the eyes is characteristic of Eales syndrome. In the worst case, this leads to temporary blindness in one eye.
It is very important to visit your ophthalmologist regularly, who can decide on further measures. There is an urgent need for action in an emergency. This is especially true in the case of severe clouding of vision. The emergency doctor must be called here.
In everyday life it is very important that the recommendations and instructions of the attending physician are followed. It is often possible to lead a largely normal life for a long time.
Especially in the case of rare diseases, such as Eales syndrome, it can be very helpful to exchange ideas with other sufferers. This can be done in a self-help group on site or in an internet forum. By exchanging ideas with others, stressful situations can be endured more easily, experience can be gained and passed on, and valuable friendships can be made with those affected.
It doesn’t even have to be a group in which only patients with Eales syndrome come together. Such groups are rather rare. Retinal self-help could be an important point of contact.