Scleritis is an inflammation of the sclera of the eye that, if left untreated, can cause loss of visual acuity. The peak age of the disease is between the ages of 40 and 60, and women are affected significantly more often than men.
What is scleritis?
Scleritis is a diffuse or localized inflammation of the sclera, which in many cases has a chronic course with recurrences (40 percent). Different forms of scleritis are differentiated depending on the localization and course. If the inflammation is located in the anterior region or in front of the eyeball equator, it is called anterior scleritis, while a focus of inflammation in the posterior region or behind the eyeball equator is called posterior scleritis. In addition, anterior scleritis is subdivided into necrotizing forms with whitish necrotic areas, diffuse scleritis that usually affects the eyeball in sectors, and nodular subtypes with displaceable reddish-blue nodules. As a result of the inflammation, edematous (fluid storing) scleral swellings manifest with marked tenderness of the affected eye, which in combination with eyelid edema and chemosis may cause visual impairment. The pressure pain characteristic of scleritis may radiate to each affected side of the face.
Causes
In many cases, the cause of scleritis cannot be determined (idiopathic scleritis). In about 50 percent of cases, the disease may be associated with underlying systemic diseases such as rheumatic impairments (including collagenoses such as periarteritis nodosa or lupus erythematosus, chronic polyarthritis), Crohn’s disease, gout, or autoimmunologic diseases. In particular, scleromalacia perforans (necrotizing scleritis) is often associated with marked rheumatoid arthritis. Similarly, infectious diseases such as tuberculosis, herpes zoster, or syphilis may cause scleritis. Furthermore, in some cases, keratitis (inflammation of the cornea) or iridocyclitis (inflammation of the iris involving the ciliary body) may cause secondary inflammation of the sclera. Rarely, scleritis is caused iatrogenically by medical intervention.
Symptoms, complaints, and signs
Scleritis is initially manifested by typical signs of inflammation such as redness, pain, and itching in the area of the affected eye. Characteristic is constant tearing of the affected eye, associated with visual disturbances such as veil vision or double vision, although the disturbances are not severe and usually pass quickly. As a result of the inflammation and the persistent lacrimation, a swelling forms which can be seen externally. In addition, reddish-blue nodules may appear in the area of the eye. In addition, visual acuity may temporarily decrease if the symptoms are associated with eyelid edema or chemosis. In severe cases, scleritis can lead to the development of staphylomas. Scars often form or there are permanent limitations in vision. Symptoms of scleritis appear within a few hours to days. Typically, visible redness occurs shortly after the triggering event. This is followed by the characteristic swelling, accompanied by a sudden decrease in vision. If the disease is treated directly, the symptoms usually subside quickly. Untreated scleritis can be chronic and, in the worst case, lead to blindness of the affected eye.
Diagnosis and course
Diagnosis of scleritis is generally based on the symptoms characteristic of the disease, particularly the marked pain on pressure. During physical examination with a slit lamp, a thickening of the sclera can be detected in the slit image as a result of the swollen scleritic area. In addition, the slit image can be used to determine the extent of inflammation and the clinical form of scleritis. Sonographic (ultrasound) imaging of scleral changes is possible. Scleritis should be differentiated from conjunctivitis, corneal inflammation and episcleritis. Furthermore, if scleritis is confirmed, a possible underlying systemic disease should be investigated. Prognosis and course of scleritis strongly depend on the specific form.For example, affected individuals with anterior diffuse scleritis (9 percent) have a much lower risk of visual loss than those with nodular (25 percent), necrotizing (75 percent), or posterior scleritis (80 percent). Underlying autoimmunologic diseases also have an unfavorable effect on prognosis.
Complications
In some cases, scleritis may occur in association with corneal inflammation or iris dermatitis. This can exacerbate the original symptoms. The edema and swelling may become inflamed and cause further complications. In extreme cases, the infection spreads to other parts of the body. Very rarely – for example, if the patient is already weakened due to another illness – blood poisoning can occur, which may be fatal. In cases of prolonged illness, eye pain can also cause psychological problems. The same applies to visual disturbances, which often place an extreme burden on young patients in particular. Scleritis therapy is usually symptom-free. However, antirheumatic drugs, anti-inflammatory drugs and other drugs that are usually prescribed can cause side effects and interactions. Occasionally, allergic reactions occur or an existing intolerance causes discomfort. In the course of a surgical procedure, the usual complications may occur: Bleeding, secondary bleeding and infections. In a severe course, loss of vision may occur in rare cases. Wound healing problems may occur after surgery. In addition, scars often develop, which may be associated with pain and a feeling of pressure in the area of the surgery.
When should you see a doctor?
When scleritis occurs, medical treatment must be sought. In the worst case, the affected person suffers a complete loss of vision due to this disease if left untreated, which is usually irreversible. For this reason, a doctor should always be consulted immediately in the case of scleritis. A doctor should be consulted if the affected person suffers from severe redness or itching of the eye. This causes a severe inflammation of the eye, which does not disappear on its own. In general, sudden visual complaints indicate scleritis and should be investigated by a doctor if they occur without a specific reason. In many cases, those affected also suffer from double vision or veil vision and are unable to cope in their daily lives. If these complaints occur, the affected person should consult an ophthalmologist. Further treatment depends on the severity of the scleritis, so no general prognosis can be given here.
Treatment and therapy
Therapeutic measures in scleritis are based on the specific underlying disease present and aim to reduce symptoms. Systemic symptom reduction is generally achieved with nonsteroidal anti-inflammatory drugs or anti-inflammatory drugs such as flurbiprofen or indomethacin, which are administered orally and gradually phased out over several months of therapy (gradual reduction of the drug dose). The pain present in the individual can assist in determining the appropriate dose, as this serves as an indicator of the extent of inflammatory activity. Acute inflammation can be treated systemically with corticosteroids (including prednisolone) if the pain is pronounced. If this medication is ineffective or if there are pronounced side effects, the therapy can be adjusted and switched to non-steroidal immunosuppressants such as cyclosporine A, methotrexate or azathioprine. In necrotizing scleritis, immunosuppressants (especially cyclophosphamide) and initially, if necessary, peroral or intravenous steroids are used as standard. In those with rheumatoid arthritis and diffuse or nodular scleritis, nonsteroidal anti-inflammatory drugs are recommended, with a switch to methotrexate in nodular scleritis if appropriate. If there is progressive necrotization of the sclera with increased risk of bulbar perforation, surgical intervention (scleral transplantation) with peri- or postoperative immunosuppressive therapy is indicated. In addition, possible concomitant infections of scleritis should be treated with antibiotics (bacterially induced) or cold compresses and/or synthetic tears (virally induced).
Prevention
Scleritis can be prevented by consistent and adequate therapy of underlying rheumatic, autoimmunologic, or infectious diseases. In contrast, no prophylactic measures exist against idiopathic scleritis because of its unknown etiology.
Follow-up
Affected individuals usually have few and also limited measures of follow-up care available for scleritis. For this reason, patients should seek medical attention at the first symptoms and signs of the disease to prevent further complications. As a rule, the disease cannot be cured on its own, so that the affected person is always dependent on medical examination and treatment. The earlier a doctor is consulted, the better the further course of the disease usually is. This disease does not need to be treated permanently, although regular checks and examinations by a doctor are nevertheless very important. Especially the kidneys and the urinary tract should be checked regularly. Likewise, regular blood analyses should be performed. In some cases, scleritis can lead to infections or inflammations of the urinary tract or kidneys, so that treatment with antibiotics is necessary. Regular medical check-ups are also very important. If the disease is treated by surgery, the affected person should take it easy after the procedure and especially protect the affected region.
What you can do yourself
In the case of scleritis, medical treatment is needed first. In addition, a whole range of self-help measures can be taken to support the medical or surgical treatment and thereby contribute to a rapid recovery. The first step is to treat the individual symptoms on their own. For skin redness and infections, compresses and cooling help. If necessary, a diet can reduce the strain on the skin. It is advisable to avoid dairy products and spicy foods. Alcohol and nicotine are also considered harmful and should be avoided. Any accompanying infections are treated by means of cold compresses. In consultation with the doctor, so-called synthetic tears can also be used. In any case, treatment with the strong agent must be carried out under medical supervision in order to avoid complications and to be able to clarify queries quickly. Scleritis is a serious disease associated with various symptoms and complaints. Home remedies and self-help measures can support medical therapy, but not replace it. If the above tips do not bring improvement, the family doctor must be informed so that medical treatment can be adjusted.
