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Uveitis and Scleritis

Uveitis and Scleritis are uncommon conditions but when they occur they can be devastating. It is important that a correct diagnosis is made, and that the possible causes are looked for, and that a treatment plan is worked out. Inflammation in the eye requires a careful approach.

What is Uveitis

Uveitis is an inflammatory problem in the tissues within the eye. This means inflammation deeper to the surface. Other names used to describe problems within the eye include:

Iritis or anterior uveitis: Inflammation of the front compartment of the eye, namely the iris and ciliary body. This is usually characterised by the presence of inflammatory cells in the anterior chamber of the eye. These cells are visible to the ophthalmologist examining a patient through a slit lamp (the microscope used to examine patients’ eyes)

Posterior uveitis: Inflammation in the back part of the eye, namely in the vitreous gel, the retina and/or the choroid. Depending on the exact part of th posterior part of the eye involved, other terms used may include retinitis, choroiditis, pars planitis, intermediate uveitis, retinal vasculitis.

Pan uveitis: This is a term used to describe inflammation of both the front and the back parts of the eyes, including the optic nerve in some cases.

Symptoms of Uveitis

Depending on the type of uveitis, it may or may not have symptoms. Most patients are symptomatic, and complain of various combinations of discomfort and/or visual problems, developing over days to several months.

Symptoms of discomfort usually develop rapidly and include photophobia (eye pain when exposed to light), pain in the eye, tenderness to touch, and pain when moving the eye or attempting to “focus in”.

Visual symptoms may include foggy or blurry vision, floaters (moving black spots), or distortion of shapes. Visual symptoms may develop at the onset of uveitis or may take several months to be noticed.

Diagnostic Testing

The medical approach to diagnosing these problems includes several levels:

1. The specialist examines the patient and defines the extent and severity of the eye problem.
2. An attempt is then made to find a cause and look for other health problems which may underlie the eye problem.
3. A decision whether treatment is required, and what treatment to use.

Typically, patients with ocular inflammation undergo a clinical assessment (describing their symptoms and answering relevant medical history questions). This is followed by examination of the eyes and, when relevant, a general medical physical examination. In many, but not all cases, further workup is required including laboratory and imaging tests. The need for such tests is determined by the specialist based on his/her clinical impression.

After conducting a medical assessment and laboratory workup, the specialist may arrive at one of the following conclusions:

1. The condition is limited to the eye and is the result of a specific, treatable problem (for instance, an infection with toxoplasma or a herpes virus)
2. The condition is limited to the eye and is the result of an immune disorder of an unknown cause (for instance, vasculitis of the retina)
3. The condition is part of a systemic problem (involving other organs than the eye), and is the result of a specific, treatable problem (for instance an infection with tuberculosis or the herpes virus)
4. The condition is part of a systemic immune disorder of unknown cause (for instance sarcoidosis, ankylosing spondylitis, Behcet’s disease). While many of these conditions have specific medical names, there is no clear understanding why they occur, and they are treated using similar general principles and similar drugs. However, given their potential effect on other organs, there is often a need for a team of doctors to be involved, most commonly an ophthalmologist and a rheumatologist.

Practically speaking, in many patients who have inflammatory eye diseases such as uveitis or scleritis no cause is found for the problem. In others, the disease is characteristic and has a medical name (for instance, sarcoidosis) and may involve other parts of the body, but there is no understanding why it occurred and what caused it.

Treatment of Uveitis

This is a very broad question and the answer depends on the type of uveitis, and the cause, if known. Each patient has different problems and needs, and the treatment is decided on based on the individual circumstances.

Treatment may include:

*Local anti inflammatory treatment to the eye/s in the form of eye drops.
*Local anti inflammatory treatment to the eye/s in the form of injections to the vicinity   of the eye or to the eye.
*Oral medications, including corticosteroids (cortisone type drugs) antibiotics, antiviral tablets, and/or drugs which suppress the immune system.
*Local medical and surgical treatment of possible eye complications of uveitis, such as cataract, glaucoma or epiretinal membrane.

Common Questions

Q: What can I do to avoid “flare ups” of my condition? 

A: This is one of the most commonly asked questions, and the answer is far from satisfying. It would be fair to say that, for most of these conditions, there is no understanding of the reason they occur at a given time to a given person. There is therefore not a lot one can do to reduce the chance of a recurrent disease. Changing your diet, exercise patterns, environment, stress levels etc are not known to make a difference.

Q: Will I go blind from uveitis?
A: In the vast majority of cases, the answer is NO. Uveitis may inflict considerable damage on the eye/s and may rarely cause blindness. However, if diagnosed correctly and treated in a timely manner, many of its blinding complications can be avoided or treated and controlled. There is an ever increasing arsenal of drugs and techniques to battle complications of uveitis, such as macular oedema, cataract or glaucoma.

Like with any disease, there is a range of severity, and most patients with uveitis do not have a severe, vision threatening condition. Similarly, in a very small minority of patients, the condition is very severe and even aggressive therapy cannot prevent severe loss of vision. Luckily, most patients at this day and age manage to lead a normal lifestyle, work, study and drive, despite suffering from uveitis and similar conditions.

Q: Will it ever go away?
A: It certainly may, but in a very unpredictable manner. Some patients have a disease which comes and goes in short-lived episodes (“flare ups”). Others have a chronic disease which is active for many months or years. In both instances, the disease may “run its course” eventually, and become inactive, not requiring any more treatment. The challenge is to protect the eyes from the effects of uveitis and to minimise the damage caused until the disease becomes inactive.

Specific Uveitis Questions

Eye infections causing uveitis:
Herpes virus infection:
The herpes group of viruses affects many people in various ways. Viruses which belong to this group are the type 1 and type 2 herpes simplex (Type 1 most commonly causes mouth “cold sores” and type 2 causes genital herpes), as well as the zoster virus which causes chickenpox and herpes zoster of the skin (“shingles”). Another member of this group of viruses is cytomegalovirus, CMV.

Herpes viruses are one of the commonest infections affecting the cornea or the internal eye structures. They typically cause keratitis (inflammation of the cornea) with or without uveitis (inflammation of the internal eye structures). The zoster virus tends to affect the eye when the skin of the eyelids and forehead is affected by “shingles”, a common condition referred to as herpes zoster ophthalmicus.
By nature, herpes viruses tend to stay in the body for life, occasionally becoming active and causing symptoms, then becoming dormant again for unpredictable periods. When active they are capable of creating a wide range of problems, from trivial discomfort and red eye, to severe eye damage.
The diagnosis of these viral infections is usually clinical (by examining the patient and the eye). In some cases there is uncertainty about the diagnosis and laboratory testing is necessary, taking samples from the surface of the eye or from the fluids inside the eye.
The treatment of herpetic infections relies on antiviral drugs which can be delivered to the eye either by eye drops, ointments or by using oral or intravenous antiviral medications. A typical example is acyclovir. These drugs are often effective in keeping the viruses less active and limit the damage they cause to the eye. However they do not completely cure the infection and do not eliminate the virus from the body. In patients with frequent recurrences there is often a need for long term treatment with antiviral medications (often by mouth), which may be required for months or years.
Patients with herpetic eye infections may develop a range of problems in the eye as a result of the infection. Examples include scarring and clouding of the cornea, paralysis of the pupil muscle, causing a permanently dilated and poorly reactive pupil, cataract, glaucoma and high pressure inside the eye, damage to the retina and optic nerve and retinal detachment.

What is Scleritis

Scleritis is an inflammatory process affecting the white of the eye, or sclera. This tissue is tough and forms the “external wall” of the eyeball. The muscle which move the eyes attach to the sclera.
Typical symptoms of scleritis include red eye/s, severe pain in the eye, which may occur at night and wake the patient from sleep, pain when moving the eyes, or trying to read, and sometimes pain in the light or reduction in vision. Eyes with scleritis are often tender to touch.

Underlying reasons for scleritis: In many patients with scleritis, no cause is found for their eye disease and they have no other inflammatory diseases. However, various inflammatory (auto-immune) diseases may cause scleritis and in some patients scleritis is their first sign. Common examples are rheumatoid arthritis, lupus, relapsing polychondritis and Wegener’s granulomatosis (also called granulomatosis with polyangeitis). Infections may also cause scleritis, especially after injuries or surgeries on the sclera.

Severe scleritis may create permanent damage and thinning of the sclera and cornea. When this occurs, we use the term “necrotising scleritis”. These cases tend to occur more in patients with underlying immune diseases and an effort is made to identify and such conditions which may require treatment. Necrotising scleritis also has a higher potential for causing permanent visual damage.

Treatment of Scleritis

The principles of treatment are similar to those described above for uveitis. Mild scleritis often responds well to oral anti inflammatory medications such as indomethacin, ibuprofen and diclofenac. Steroid (cortisone) eye drops may also help the symptoms in some patients.
More severe cases require oral treatment with oral or intravenous cortisone type medications (e.g. prednisolone) and sometimes systemic drugs which suppress the immune system, as described above.
In many cases, injection of steroids to the inflamed area has a rapid effect in stopping the pain and redness, although recurrences may occur once the medication has absorbed and disappeared from the eye (usually after months). This treatment may avoid the need for systemic treatment and its potential side effects. However, like any other local steroid treatment to the eye, it has a risk of causing high pressure in the eye (glaucoma) and cataract.

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