Eales syndrome is eye vasculitis associated with retinal damage, vitreous hemorrhage and loss of visual acuity. The causes of the disease are still unclear and may correspond to autoimmune processes on tuberculosis phenomena of different contexts. The symptomatic treatment of the syndrome encompasses medical and ophthalmological steps.

What is the Eales syndrome?

At the onset of disease patients with Eales syndrome often show no manifest symptoms. For example, premature symptoms of the disease include a blurred vision that results in a painless loss of vision.
© PATTARAWIT - stock.adobe.com

A so-called vasculitis is an inflammatory disease that affects the blood vessels. Vasculitides can occur in almost every tissue and have different causes. The Eales syndrome is attributed to the Vasculitiden and corresponds to an eye disease, which leads to retinal damage with loss of vision and vitreous hemorrhage.

The disease is named after its first writer Henry Eales. The English ophthalmologist described the phenomenon at the end of the 19th century. In medical literature, the disease is also known as retinopathy of retinopathy or retinal periphlebitis.

Sometimes the phenomenon also refers to idiopathic perivasculitis of the retina or idiopathic retinal pervaculitis. The prevalence of eye disease is unknown. Mainly male adults are affected by Eales syndrome. The average age of onset is between 20 and 30 years of age.

causes

The causes of the Eales syndrome are so far as little understood as the genesis of the disease. Since no familial accumulation has been observed in the previously documented cases, 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 association with the vasculitis is at least explaining the reasons for the loss of vision during the syndrome. Inflammation of the supplying vessels form the initial stage of the syndrome. This inflammatory stage is associated with periphlebitis of the peripheral retinal areas. At the ischemic stage sclerotherapy of the retinal veins occurs, which in the subsequent stage of proliferation into neovascularization with bleeding passes.

These processes lead to retinal detachment. Scientific suspicions suggest that the syndrome is a multifactorial genesis of autoimmunity, Mycobacterium tuberculosis, oxidative stress, and possibly coagulopathy. As a rule, the disease occurs spontaneously and without inflammatory or traumatic conditions.

Symptoms, complaints and signs

At the onset of disease patients with Eales syndrome often show no manifest symptoms. For example, premature symptoms of the disease include a blurred vision that results in a painless loss of vision. Visual acuity is rarely worse than 20/200.

Different complaints indicate vascular damage: Rubeosis iridis occurs in the anterior chamber of the eye and, in isolated cases, glaucoma caused by neovascularization. The posterior chamber of the eye shows vitreous hemorrhage as well as traction aortic.

In later stages, proliferative retinopathy often occurs with retinal hemorrhage. Periphlebitis Retinae is one of the most common symptoms. Central nervous system symptoms have been described in isolated cases as complications. These symptoms are mainly emotional disorders, speech disorders and loss of exercise.

Diagnosis & History

The diagnosis of the Eales syndrome is under the differential diagnostic exclusion of diseases such as sarcoidosis, the closure of retinal vein branches and other proliferative retinal diseases. The diagnostic tool of choice is usually a fluorescein angiography of the eye fundus.

In addition to this diagnostic test, a sonography can be done, which is especially helpful in retinal detachment and vitreous hemorrhages. Different authors also recommend an analysis of blood clotting. Basically, there is a rather favorable prognosis for patients with Eales syndrome. Complete blindness has been documented only in isolated cases.

Nonetheless, the course varies from person to person. A complete regression is conceivable, but just as conceivable is the progression to retinal detachment. Recurring complications have occurred in isolated cases.

When should you go to the doctor?

Since the Eales syndrome can lead in the worst case to a complete blindness, it should be carried out in any case a study at the doctor. Self-healing does not occur in this disease. As a rule, the doctor should be consulted if the person concerned has a blurred vision or if there is a general deterioration in vision. This deterioration usually occurs very suddenly.

Speech disorders or paralysis also point to the Eales syndrome and must be examined in any case. Likewise, through the Eales syndrome restrictions may occur in the movement, which must also be examined by a doctor. First and foremost, the ophthalmologist can be consulted for this disease. The sooner the disease is diagnosed, the higher the likelihood of a positive disease course. Since the symptoms of the illness can recur even after a successful treatment, the person concerned should also take part in regular examinations after the treatment.

Treatment & Therapy

Causal therapy is not available for patients with Eales syndrome until now. The reason for this lies in the insufficient elucidation of causative factors. Causal therapies target the primary cause of a disease to stem the disease process.

By eliminating the cause, the individual symptoms are also permanently established with causal therapeutic steps. Although the idiopathic Eales syndrome does not provide a causal starting point, at least symptomatic treatment approaches are available. Symptomatic treatment steps depend on the symptoms in the individual case and fall in the case of Eales syndrome mainly depending on the stage.

For example, at the stage of perivasculitis, patients receive corticosteroids. This conservative drug treatment may correspond to a systemic or peri-ocular form of therapy. Systemic administration means the body-encompassing mode of action. The corticosteroids are administered perorally or via an infusion in this context and thus act on the entire organism via the bloodstream.

More gentle and targeted is the peri-ocular administration at the site of the pathological event. Since it is suspected that autoimmune processes are involved in the inflammatory disease, immunosuppressive drugs such as azathioprine or cyclosporin may be used as an experiment. The immune system of patients is attenuated by these means.

Due to the possibly involved tuberculosis factors some scientists recommend an additional tuberculostatic therapy. However, this approach is considered controversial. In the proliferative stage photocoagulation is the therapy option of choice. Also, vitreous retinal surgery can be used, especially in long-lasting vitreous hemorrhages or imminent adhesions with the risk of retinal detachment.

Outlook & Forecast

The course of the Eelessyndrom is very different. It can lead to both regression and progression of the disease. A causal therapy does not exist so far. It can only be treated symptomatically. Often there is a loss of vision. Nevertheless, complete blindness is very rare even in untreated individuals.

Problems occur when vitreous bleeding and retinal bleeding occur over a long period of time. Retinal detachments also occur. If the vitreous hemorrhage lasts more than three months or there is a threat of retinal detachment, vitreous retinal surgery becomes necessary. The constant neovascularization causes growths, which can lead to glaucoma in addition to retinal detachment. Without treatment threatens the loss of sight here.

Although all treatments can alleviate the most severe symptoms, they do not affect the course of the disease. Since the causes of an Eelessyndroms are not yet completely cleared, also no causal therapy was found. As already mentioned, the symptoms can either completely regress or continue to progress, with or without treatment.

As the disease progresses, vascular degeneration and neovascularization alternate with each other. During neovascularization (proliferation) new cells are constantly being formed which lead to proliferation. In a proliferation-induced risk of retinal detachment, photocoagulation is recommended as the therapy to fully retina the retina.

prevention

To prevent the Eales syndrome is not so far, since the causes are still considered idiopathic. Only when the causative factors involved have been finally clarified, preventive approaches are available.

aftercare

The measures or possibilities of follow-up are very limited in the case of Eales syndrome. In any case, the affected person is dependent on a quick diagnosis with a subsequent treatment, since otherwise, if left untreated, complete loss of vision may occur. This can be irreversible and thus can no longer be treated. Therefore, in the foreground of the Eales syndrome is the early detection of the syndrome.

If the first signs or symptoms of this disease occur, an ophthalmologist should be contacted immediately. In most cases, patients with Eales syndrome need medication to relieve their symptoms. It should be paid to a regular intake with a proper dosage.

In case of ambiguity or questions, a doctor should always be contacted first to avoid false assumptions. In children, parents should check the correct intake. Furthermore, regular checks by an ophthalmologist are very important to control the stage of the Eales syndrome. In some cases, surgery may be necessary. After such surgery, the eye should be particularly well protected, usually with a bandage worn around the eye.

You can do that yourself

The Eales syndrome is a relatively rare eye disease whose causes are still largely unknown. Everyday life with this disease varies greatly, depending on how severe the symptoms are. Characteristic of the Eales syndrome is the bursting of blood vessels in the eyes. In the worst case, this leads to the temporary blindness of an eye.

It is very important to visit the ophthalmologist regularly, who can decide on further measures. In an emergency there is an urgent need for action. This is especially true in severe cloudiness of vision. Here the emergency doctor must be called.

In everyday life, it is very important that the recommendations and instructions of the attending physician are observed. So it is often possible for a long time to lead a largely normal life.

Especially with rare diseases, such as the Eales syndrome, it can be very helpful to interact with other stakeholders. This can be done in a self-help group on site or in an internet forum. By sharing with others, stressful situations can be more easily endured, experiences gained and shared, and valuable friendships made with those affected.

It does not even have to be a group in which only patients with the Eales syndrome come together. Such groups are rather rare. An important contact person could be the retina self-help.

Tags: 
  • pathogen 
  • parasite 
  • news 
  • Diseases 
  • pregnancy 
  • Top