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Uveitis Frequently Asked Questions

  1. What is Uveitis and what are some of the symptoms?
  2. What is the difference between local and systemic related Uveitis?
  3. Are there different kinds of Uveitis ?
  4. What are the presenting signs and symptoms of the different kinds of Uveitis?
  5. What are the general causes of Uveitis?
  6. How is Uveitis diagnosed?

What is Uveitis and what are some of the symptoms?

Uveitis is an inflammation in or around the eyeball. It can be acute, episodic, chronic, or recurring. Typical signs and symptoms include redness, pain, light sensitivity, and blurred or distorted vision. Uveitis can be unilateral affecting one eye or bilateral in both eyes. Less commonly seen symptoms include eye or lid swelling, sudden loss of vision, severe pain or symptoms occurring in both eyes simultaneously.

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What is the difference between local and systemic related Uveitis?

Local related Uveitis affects only the area in and around the eye(s) and is not associated with an inflammation or infection in the rest of the body. Systemic related Uveitis is and inflammation of the eye(s) that is associated with an inflammation or infection located elsewhere in the body. It is very important to accurately diagnose the cause of Uveitis and whether it is local or systemic in its origin in order to determine an effective course of treatment.

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Are there different kinds of Uveitis?

Yes, we've already discussed local and systemic related Uveitis. Uveitis can be described base on the location or how it appears within the eye.

Classic internal ocular location descriptions:

  • Anterior Uveitis (Iridocylitis, Cyclitis, Iritis)
  • Intermediate Uveitis (Cyclitis. Pars Planitis, Anterior Vitritis)
  • Diffuse Uveitis (Pan Uveitis)
  • Posterior Uveitis (Retinitis, Choroiditis)

External eye location descriptions:

  • Conjunctivitis - inflammation of the conjunctiva
  • Keratitis - inflammation of the clear cornea
  • Episcleritis - inflammation of the vessels lying on top of the sclera and underneath the conjunctiva
  • Scleritis - inflammation of the tough outer coat of the eye

Anatomic structure descriptions:

  • Trabeculitis - inflammation of the fluid drainage mechanism in the front of the eye
  • Iritis - inflammation of the colored part of the pupil
  • Cyclitis - inflammation of the fluid producing part of the eye at the base of the iris
  • Vitritis - inflammation of the normally clear gel located inside of the eyeball
  • Retinitis - inflammation of the neurosensory retina
  • Choroiditits - inflammation of the layer between the retina and sclera that contains the blood supply to the retina
  • Vasculitis - inflammation of the veins or arteries within the eye, usually on the surface of the retina
  • Optic Neuritis - inflammation of the optic nerve

Unique Uveitis descriptions:

  • Keratin Precipitates - clumps of inflammatory cells on the back of the clear cornea
  • Pars Plana Snowbanking - layer of inflammatory cell and fibrosis inside the eye on the Pars Plana
  • Cystoid Macular Edema - focal swelling in the center part of the retina associated with reading vision
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What are the presenting signs and symptoms of the different kinds of Uveitis?

Uveitis can effect the eye with different symptoms depending on the location of the Uveitis. About the only common symptom to most forms of uveitis is blurred vision. Other than that, uveitis can have some, none, or all of the following complaints:

  1. Anterior Uveitis or iritis usually develops over a period of several days of a week, with common symptoms including floaters, redness, blurred or distorted vision, light sensitivity, and eye pain. Intermediate Uveitis is usually painless and the eye is white appearing.
  2. Intermediate Uveitis can have blurred vision and floaters.
  3. Posterior Uveitis often occurs with systemic disease. It tends to develop more gradually and is often more deep-seated by the time the diagnosis is made, making it even more difficult to treat. Symptoms of Posterior Uveitis can be episodic (one to three times a year or less), recurrent (symptoms subside but relapse or recur), or chronic (symptoms are always present).
  4. Diffuse Uveitis (or Panuveitis) describes inflammation that is not localized to one area within the eye and is more generally distributed throughout the eye.
  5. Unilateral Uveitis occurs in only one eye, while Bilateral Uveitis occurs in both eyes. Uveitis can even behave in varying patterns: episodic, recurrent, bilateral but alternating (affecting both eyes over time but not both at the same time).
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What are the general causes of Uveitis?

Infections, autoimmune problems, and inflammatory responses are the most common underlying causes of Uveitis.

INFECTIONS - Bacteria, fungus, or viruses can cause infectious Uveitis. Infectious forms of Uveitis can be cured if the appropriate diagnosis is made and the correct antibiotics or antiviral agents are prescribed. Unfortunately, infections are a frequently overlooked cause of Uveitis.

AUTOIMMUNE - Autoimmune Uveitis is inflammation in the eye that results from the body's immune system attacking the cell tissues of the eye. Autoimmune Uveitis is probably the most common cause of Uveitis. Diagnoses often seen in autoimmune Uveitis include rheumatoid arthritis, Behcet's syndrome, and HLA B-27 (a tissue antigen).

INFLAMMATORY - Some cases of Uveitis have underlying causes which cannot be more specifically diagnosed but are treated similar to autoimmune Uveitis. Examples of inflammatory Uveitis are Fuchs' heterochromic iridocyclitis, pars planitis and sarcoidosis.

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How is Uveitis diagnosed?

Because it is so important to make an accurate diagnosis to treat Uveitis, the initial Uveitis visit is very through and takes several hours to complete. The complete evaluation of Uveitis may require several visits and includes many of the following components:

HISTORY - This includes the ocular and medical history of the patient, how the Uveitis has presented in the past, and how it has responded to treatment, as well as other information that may give clues as to how the disease is related to other parts of the body. An accurate history is considered to be one of the most important aspects of the exam, because it is often the single most critical element in making the correct diagnosis.

REVIEW OF SYSTEMS - This information is usually a series of questions and is designed to uncover symptoms in other parts of the body that are actually related to the disease process in the eye and may provide clues to help determine the cause of the Uveitis.

EXAMINATION - Complete ocular examination including dilated examination with limited general physical examination as required (i.e. skin, joints, mouth). The emphasis is on determining the anatomic location and systemic associations, if any, of the Uveitis.

DIFFERENTIAL DIAGNOSIS - Based upon the history, review of systems, and examination a differential diagnosis or listing of the most likely possible reasons for the Uveitis is refined. These are then systematically evaluated and ruled in or out as potential causes for the Uveitis. This stepwise investigation usually requires specialized testing to prove or disprove possible causes.

LABORATORY TESTING - The differential diagnosis leads to ordering specific tests to determine if a possible diagnosis is in fact, present or not. Shotgunning lab tests rarely results in a diagnosis. There are no "typical lab tests" as there are no really "typical Uveitis patients". Each evaluation requires careful examination, clinical suspicion, an experience to determine what lab tests may be most beneficial. Consider the following very limited example:

A patient has a unilateral choroidal granuloma (posterior inflammatory nodule). The differential diagnosis suggests that among other possibilities, sarcoid or tuberculosis could be present. Specific tests such as an erythrocyte sedimentation test, serum lysozyme, angiotensin converting enzyme, tuberculosis skin testing with control, and chest x-ray should be considered. More specialized testing such as calcium levels, pulmonary function testing, gallium scan, tissue biopsy may be required to confirm earlier positive tests. Testing this patient for autoimmune problems such as rheumatoid arthritis, multiple sclerosis, or HLA B-27 tissue antigen would not likely be productive and should not be ordered.

SPECIAL STUDIES - Ophthalmic studies such as visual field testing, ocular fundus photography, fluorescein angiography, indocyanine green angiography. Other evaluations may include, magnetic resonance imaging, CT scanning, lumbar puncture, nuclear studies (gallium scan).

SPECIALTY CONSULTATION - Treatment of severe or complicated Uveitis cases requires a coordinated team approach. Consultants in rheumatology, infectious disease, hematology-oncology, glaucoma, cornea may be needed to assist in the diagnosis, suggest possible courses of management or to initiate and support in monitoring medical management.

THERAPEUTIC TRIAL IN COMPLICATED CASES - In some difficult cases of Uveitis a specific diagnosis may be suspected but not provable by usual means, or two differential diagnoses are equally likely and can not be excluded. In cases like those and if the situation warrants due to risk of vision loss or disease progression then medication can be prescribed as a therapeutic trial. An example situation would be a patient with a possible diagnosis of tuberculosis versus sarcoidosis. Both diagnoses are severe, potentially blinding ocular inflammations. One (tuberculosis) is treated with specific and potentially toxic antibiotics (INH, PZA, ethambutol, etc.) that can cure TB related uveitis and not treat but probably not worsen sarcoidosis. The other (sarcoidosis) is treated with anti-inflammatory agents (corticosteroids, rarely cyclosporine A) that can effectively treat sarcoidosis but very likely might worsen tuberculosis. In this simplified but representative example there are three fairly complicated options:

  1. Do nothing since:

a. TB might not be present and the treatment for TB can have serious side-effects.

b. Sarcoid might not be present and corticosteroids have many serious side-effects.

c. If you treat for TB you could be wrong and if you treat for sarcoid you could be wrong too.

  1. Treat with corticosteroids for a presumed diagnosis of sarcoidosis as the likelihood of TB is probably remote and therefore the risk of the condition worsening is remote.
  2. Treat for a presumed diagnosis of tuberculosis weighing the drug toxicity (liver inflammation, optic nerve damage) against the risk of visual loss if untreated or risk to the patient if TB is missed. Treatment is usually continued for two months to determine if the uveitis will respond. A decrease in the inflammation suggests that TB is the causative organism and therapy can be continued. No response by two months indicates that TB is unlikely and perhaps sarcoidosis should be more bly considered.

All three options have pros and cons.

Option one is problematic since doing nothing may not cause direct harm but would probably result in the patient's condition becoming worse over time. If the patient cannot tolerate the medications for either condition or is not a medical candidate for treatment, option one might be the only choice. Option one is the least effective choice.

Option two is problematic since there is a risk of worsening TB if TB is the actual diagnosis. In clinical practice, Sarcoid will usually respond relatively rapidly to appropriate doses of corticosteroids unlike the general pattern of TB which would improve somewhat initially (due to anti-inflammatory effects) but then gradually show a worsening pattern (steroid resistance). If the patient demonstrates a clinical pattern suggesting rapid resolution then sarcoid appears to be correct but if the pattern is one of relative steroid resistance and worsening, then TB is more likely. Options two is an inelegant solution.

Option threeis problematic due to the toxicity of the anti-TB antibiotics. Antibiotic therapy for TB uveitis usually takes at least two months to show a clear response. Options three would require TB antibiotics for a two month observation period and, if the uveitis responded within that time frame, then the uveitis could be reasonable concluded to be due to TB. Anti-TB therapy would be continued. If there was no response after two months of adequate therapy for TB then TB can be reasonably excluded and steroid therapy for sarcoid begun. In choice three the risk of worsening sarcoid by a two month delay in treatment is fairly low relative to the benefit of not missing a potential case of tuberculosis. Option three poses a systematic approach to differentiating among two diagnostic choices with the least relative risk to the patient and best chance of isolating a successful treatment for the uveitis.

All three options have relative risks and benefits. Any of the three options might be right for a specific patient under specific circumstances. There are no typical uveitis treatments because there are no typical uveitis patients. Therapeutic trials should be carefully considered and supported by clinical and laboratory data. The rational, risks, and benefits of a therapeutic trial should be fully explained and understood. Trying random medications hoping to get lucky is foolhardy.

SURGICAL BIOPSY - Tissue samples are occasionally needed to establish or confirm a diagnosis. Biopsy sites are taken from active disease sites in or around the eye (conjunctiva, lachrymal gland, sclera, choroid, retina) and occasionally from specific systemic locations such as the parotid gland or skin lesions.

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Copyright © 2000 Retina and Uveitis Consultants of Texas, P.A. - Last modified: May 1, 2003