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NORD gratefully acknowledges Ramisha Ali, Doctor of Pharmacy Candidate, NORD Editorial Intern from the Keck Graduate Institute, and Mylene Manalo, PharmD, Assistant Professor of Clinical and Administrative Sciences, Keck Graduate Institute School of Pharmacy and Health Sciences, for assistance in the preparation of this report.
Uveitis is a general term that refers to inflammation of the part of the eye known as the uvea. The uvea is a relatively thick, strong layer of fibrous tissue that encloses and protects the eyeball. It contains many blood vessels and can cause inflammation in neighboring structures of the eye such as the retina, vitreous, optic nerve head and retinal vessels. The uvea consists of three parts: the iris, ciliary body and choroid.
There are four types of uveitis, classified according to the part of the uvea that is affected. Anterior uveitis, which affects the front part of the eye, is also sometimes called iritis since the iris is part of the front of the eye. Almost 80% of cases of uveitis affect the anterior portion of the eye. Intermediate uveitis, also known as pars planitis or cyclitis, refers to inflammation of tissues in the area just behind the iris and lens of the eye. Posterior uveitis, also known as choroiditis, refers to inflammation of the choroid, the back part of the uvea. Posterior uveitis may affect the retina and/or the optic nerve and may lead to permanent loss of vision. Panuveitis refers to inflammation throughout the eye and does not refer to a specific area.
Posterior uveitis is the rare form of the disorder and is the type of uveitis most associated with loss of vision. Uveitis can affect one or both eyes and it affects people of all ages, including children. Posterior uveitis can cause further complications if it is not treated, such as blindness.
People with certain genes may be more likely to develop posterior uveitis. Patients who have a weakened or impaired immune system (immunocompromised) such as those with HIV or AIDS are at higher risk for viral posterior uveitis. People with a normally functioning immune system may also develop viral posterior uveitis. Cigarette smoking has been associated with a harder to manage disease.
Some people do not experience symptoms; however, some who experience them may have a sudden onset and get worse quickly.
Symptoms that people may experience if they have posterior uveitis include:
Floaters are small specks, flakes or clouds that move through the field of vision and decrease vision. While anterior uveitis often causes eye pain and redness, light sensitivity and blurred vision, the symptoms of posterior uveitis are more subtle. Uveitis can lead to other complications including glaucoma, cataracts or retinal detachment. Of note, 50% of patients have reduced vision and 10-15% have complete vision loss. Early detection and treatment is important to reduce the risk of permanent vision loss.
Uveitis can be caused by a person’s immune system, infections, tumors, bruising, eye injury or exposure to toxins. Posterior uveitis is inflammation of the uvea due to the body’s response to various factors such as microorganisms, toxins and damaged tissue. Inflammation may cause redness, swelling and heat in a particular area of the eye. This reaction can trigger an immune response and lead to the destruction of certain tissues and cause white blood cells to collect in that area of the eye.
Posterior uveitis can have infectious or noninfectious causes. Infectious causes include bacterial, fungal, parasitic and viral infections. Noninfectious causes include immunologic problems, allergies, malignancies and unknown causes. Infectious causes include herpesviruses, measles, rubella and arboviruses such as dengue, West Nile and chikungunya virus. Oftentimes, viruses can remain latent in the body and can lead to serious complications and vision loss over time. The most important risk factor for viral posterior uveitis is being in an immunocompromised state, such as with HIV and AIDS.
Posterior uveitis can be a result of associated autoimmune disorders, infections and/or trauma. Some of the disorders that can be associated with posterior uveitis are Behcet’s syndrome, ankylosing spondylitis, Lyme disease, sarcoidosis and psoriasis. The more common causes include sarcoidosis, syphilis and tuberculosis. Among children, the disorder is frequently associated with juvenile rheumatoid arthritis.
Posterior uveitis occurs as an isolated condition or as part of other diseases that affect multiple body systems. This condition affects males and females in equal numbers. It can strike at almost any age, but usually begins between the ages of 30 and 40. According to one estimate, posterior uveitis occurs in 18/100,000 people (2020).
An ophthalmic exam using direct visualization of the inflammation and or white blood cells (leukocytes) can show the vitreous humor, which is a clear jelly-like substance behind the lens. Patients should be asked about their medication history and additional laboratory tests may be performed. Lab tests are done to check if the symptoms are caused by immunological or infectious conditions. Blood studies for infectious agents such as herpes virus, toxoplasmosis, toxocariasis and spirochetes are helpful. Chest x-rays may detect sarcoidosis or tuberculosis. If systemic or central nervous system involvement is present or large cell lymphoma is suspected, neuroimaging studies and lumbar puncture may be used.
Eye exams that may be performed include:
Classification systems in uveitis are based on:
Eye doctors may use the Standardization of Uveitis Nomenclature (SUN) guidelines to diagnose this condition.
Treatment
It is important to identify and treat an underlying infection or immunological disorder before initiating other treatment.
Corticosteroids
Corticosteroids, also abbreviated as “steroids,” are considered the gold standard for treatment. Corticosteroids resemble cortisol (stress hormone) which work by reducing the activity of the immune system by decreasing inflammatory chemicals and migration of the white blood cells to the inflamed area. Thus, steroids are able to lower the amount of inflammation, redness and itching. Giving steroids on a long-term basis can cause changes in the blood cell counts and hormone levels. For example, corticosteroids can reduce the amount of white blood cells that generally stick to the blood vessels or are in the circulation, which can lead to greater infection risk. In addition, fluctuations in hormone levels upon long-term use of systemic corticosteroids can increase the risk of increased pressure in the eye (glaucoma) and cataracts, so the patient must be followed closely.
There are three different classes of steroids that can be given.
Periocular steroids
Intravitreal steroids
Systemic steroids
Long term safety concerns of taking steroids have led to the development of other therapies.
Immunomodulator Therapy
Overtime, if a patient stops responding to systemic steroids and needs to continue therapy, off-label medications may be prescribed. Immunomodulator therapies can help lower inflammation in the body and help suppress the immune system.
Immunomodulatory medications used for posterior uveitis:
Biologic Response Modifiers (BRM)
These medications are bioengineered molecules that inhibit the activity of the immune response. They target specific cytokines, interleukins, proteins and analogues. BRM are also used if the patient is unable to take other treatments.
Biologic response modifiers used for certain posterior uveitis conditions include:
Ocular Gene Therapy
Ocular gene therapy works by injecting small amounts of adeno-associated virus (AAV) and lentivirus which act as vectors. These vectors have either direct or indirect anti-inflammatory properties.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
The National Eye Institute is currently sponsoring clinical trials for posterior uveitis. Two of these studies involve the use of a monoclonal antibody as a treatment for posterior uveitis. Monoclonal antibodies are genetically engineered proteins designed to affect a specific target or biochemical reaction within the body. The monoclonal antibody daclizumab is being studied because it inhibits a specific chemical reaction needed by lymphocytes to produce inflammation. For information about these and other current trials, visit the ClinicalTrials.gov web site or contact the NIH Patient Recruitment Office (see above).
TEXTBOOKS
Thomas AS. Noninfectious Intermediate, Posterior, and Panuveitis. In: Lin P, ed. Uveitis. Current Practices in Ophthalmology. Springer; 2020:43-99. doi:10.1007/978-981-15-0331-3_3
Wade NK, Seto RJ. Posterior Uveitis. In: The NORD Guide to Rare Disorders, Philadelphia: Lippincott, Williams and Wilkins, 2003:666.
JOURNAL ARTICLES
Singh RB, Sinha S, Saini C, Elbasiony E, Thakur S, Agarwal A. Recent advances in the management of non-infectious posterior uveitis. Int Ophthalmol. 2020;40(11):3187-3207. doi:10.1007/s10792-020-01496
Lee JH, Agarwal A, Mahendradas P, et al. Viral posterior uveitis. Survey of Ophthalmology. 2017;62(4):404-445. doi:10.1016/j.survophthal.2016.12.008
Sudharshan S, Ganesh SK, Biswas J. Current approach in the diagnosis and management of posterior uveitis. Indian J Ophthalmol. 2010;58(1):29-43. doi:10.4103/0301-4738.58470
Menezo V, Lau C, Comer M, et al. Clinical outcome of chronic immunosuppression in patients with non-infectious uveitis. Clin Experiment Ophthalmol. 2005;33:16-21.
Chen CS, Robertson D, Hammerton ME. Juvenile arthritis-associated uveitis: visual outcomes and prognosis. Can J Ophthalmol. 2004;39:614-20.
Murphy CC, Greiner K, Plskova J, et al. Neutralizing tumor necrosis factor activity leads to remission in patients with refractory non-infectious posterior uveitis. Arch Ophthalmol. 2004;122:845-51. Erratum in: Arch Ophthalmol. 2004;122:1336.
Lobo A, Barton K, Minassian D, et al. Visual loss in sarcoid-related uveitis. Clin Experiment Ophthalmol. 2003;31:310-16.
Toker E, Kazokoglu H, Acar N. High-dose intravenous therapy for severe posterior segment uveitis in Behcet’s disease. Br J Ophthalmol. 2002;86:521-23.
INTERNET
Posterior Uveitis | Winchester Hospital. Last reviewed November 2019.https://www.winchesterhospital.org/health-library/article?id=102892 Accessed April 20, 2021.
Uveitis | National Eye Institute. Last reviewed July 2019. https://www.nei.nih.gov/learn-about-eye-health/eye-conditions-and-diseases/uveitis Accessed April 20, 2021.
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