Uveitis

What is uveitis?

Uveitis (“you-vee-eye-tis”) describes a constellation of diseases associated with inflammation inside the eye. I t is so named because those diseases affect the uvea, the “middle layer” of the eye, between the sclera (thick, white, outer covering) and the retina (thin, innermost layer of the eye).

What is uveitis?

The uvea consists of several structures within the eye; specifically, it is made up of the iris, the ciliary body and the choroid. D epending on the type and cause of the inflammation, just one, or at times all of them, may be involved. T he cornea, retina, sclera, and optic nerve may also be inflamed at times, which can sometimes be seen along with inflammation in the uvea.

Are there different types of uveitis?

Yes, there are, and the types of disease tend to be classified by anatomic location within the eye, and which structures are actively inflamed. T his is something that can be discerned by careful clinical examination.

The most common type of uveitis is anterior uveitis (also known as iritis, or sometimes as iridocyclitis), which typically affects the iris, and may at times involve the ciliary body to some extent as well.

Intermediate uveitis, also known as pars planitis (when no cause or association is identified), typically consists of inflammation within the vitreous cavity. T his is the least common subtype of uveitis. In some situations, there may be signs of inflammation both in the front of the eye as well as within the vitreous cavity, in which case the disease may be classified as simultaneous anterior and intermediate uveitis.

Snowballs withing the vitreous cavity

Other types of disease include posterior uveitis, which describes inflammation of the choroid (the network of blood vessels that lies underneath and nourishes the retina) and/or retina, and panuveitis, which involves some inflammation within each of the uveal structures (iris, ciliary body, and choroid).

There are other types of inflammation of the eyes that do not “technically” qualify as uveitis, since the primary site of inflammation does not lie within the uveal tract. H owever, the diagnostic tests and treatments for these diseases are similar to those for most types of uveitis. One example is retinal vasculitis, which describes inflammation that primarily involves the retinal blood vessels that can often have devastating visual consequences.

What is the significance of uveitis? Is this serious?

While the quoted statistics can vary, uveitis contributes to anywhere from 5-20% of preventable blindness, depending on geography. A nd even if it may not cause significant permanent visual impairment in every case, the associated symptoms can be bothersome and disruptive.

What are the symptoms of uveitis?

The symptoms of uveitis can vary, depending on which structure(s) within the eye is/are inflamed. Some of the more common ones include:

Snowballs withing the vitreous cavity
  • Light sensitivity
  • Eye pain
  • Eye redness
  • Blurred vision
  • Floaters

Because uveitis is not commonly or easily detected or diagnosed by physicians without training in eye diseases, it can be misdiagnosed or go undiagnosed at times. T he presence of any or all of the symptoms listed above should prompt consultation with an ophthalmologist.

What causes uveitis?

Uveitis can have a number of different causes and associations:

  • Viruses, e.g., herpes simplex virus (the one that causes cold sores), varicella zoster virus (chickenpox or shingles virus)
  • Bacteria, e.g., tuberculosis, Borrelia burgdorferi (Lyme disease),
  • Treponema pallidum (syphilis)
  • Parasites, e.g., T oxoplasma gondii
  • Systemic diseases, e.g., C rohn’s disease, ulcerative colitis, ankylosing spondylitis, sarcoidosis
  • Injury to the eye

However, in most cases of uveitis, and in ocular inflammatory disease in general, an identifiable cause or associated systemic disease is not found. What we do know is that it often stems from an underlying abnormality of the immune system, which causes the body to “attack” cells within the eye.

Rarely, a non-inflammatory condition may “masquerade” as uveitis, but require more extensive diagnostic testing and treatment, and also potentially have more severe health consequences. O ne of these “masquerade” syndromes is intraocular lymphoma, the diagnosis of which is often delayed due to its ability to mimic uveitis.

Is uveitis hereditary?

Is uveitis hereditary?

Most commonly, no. T here are certain inherited conditions that may be associated with generalized systemic inflammation that may also affect the eyes, but outside of these scenarios uveitis is generally not thought to be something one might potentially pass along to offspring.

How is uveitis diagnosed?

Most commonly, no. T here are certain inherited conditions that may be associated with generalized systemic inflammation that may also affect the eyes, but outside of these scenarios uveitis is generally not thought to be something one might potentially pass aWhat is most important in diagnosing uveitis is prompt and careful examination by an ophthalmologist upon the onset of new symptoms. There will often, but not always, be clear signs of inflammation inside of the eye upon close examination.long to offspring.

OCT

Additional testing may be required to assess the degree and severity of the eye disease, including multiple types of specialized photography/imaging.

Determining whether or not an underlying systemic disease or infection is causing or associated with your uveitis is another important step in a full evaluation. I t will likely require some radiologic testing (x-ray, CAT scan, MRI ) and blood work. A t times, it may even entail taking a sample of fluid from the eye for laboratory analysis, especially if a particular infectious  type of uveitis is suspected.

Identifying an underlying systemic condition, or a potentially infectious cause of one’s uveitis is important in directing therapy.

Can uveitis be treated? And if so, what kinds of treatments are available?

Yes, uveitis can almost always be treated. I f the inflammation seen inside of your eye(s) is due to or associated with a systemic infection, treating that infection will usually help treat the eye disease. T he same is usually true for inflammation due to an underlying systemic inflammatory condition such as sarcoidosis, inflammatory bowel disease, C rohn’s disease/ulcerative colitis or ankylosing spondylitis. Touching back upon the idea of a diagnostic workup for uveitis, it is important to emphasize that not all treatments for uveitis will be effective for all different types of disease, and appropriate testing can help determine the best way(s) to individualize a treatment plan.

Treatment of non-infectious uveitis typically requires the use of corticosteroids in order to achieve control of the inflammatory process. E yedrops are most commonly used, but depending on the location and severity of the inflammation—as well as whether or not both eyes are involved, which is usually the case—treatment may call for the use of:

  • Oral corticosteroids
  • Other oral or injected medications to alter the function of the immune system
  • Injection of corticosteroids and other medications into or around the affected eye(s).

Uncontrolled inflammation can be associated with vision-threatening complications in the eye, including but not limited to:

  • Glaucoma, or damage to the optic nerve (usually associated with elevated pressure inside the eye)
  • Cataract, a fogging or opacification of the lens inside of the eye
  • Neovascularization, growth of new blood vessels in various parts of the eye, which can lead to bleeding or the formation of scar tissue
  • Irreversible retinal damage.

It should also be noted that chronic use of corticosteroids may also lead to the development of cataracts or glaucoma. O ther systemic anti-inflammatory medications, when used, are intended to decrease the need for and potential long-term ocular and systemic side effects of corticosteroids.

What other treatment options are there, besides corticosteroids?

If the intraocular inflammation is not sufficiently controlled with steroids alone, or if the dose/frequency of steroid treatment is thought to carry significant ocular or systemic risks, treatment regimens may call for the use of steroid-sparing, immunosuppressive medications. The thought behind using these types of medications is similar to the reasons for using them in other autoimmune diseases (like lupus or rheumatoid arthritis); most immunosuppressive medications, when dosed properly, can decrease the rate of disease flares and decrease (and sometimes eliminate) the need for steroids and their potential side effects. Most importantly, disease control, which may be more easily achieved using these medications (often in conjunction with steroids), will decrease the likelihood of vision or eye-threatening complications from uncontrolled inflammation.

There are several classes of medications that may be used in helping to control chronic or recurrent uveitis. While all medications may potentially cause systemic side effects, these treatments are generally well tolerated. T hey do, however, require close monitoring for toxicity, and administration/prescribing of some medications may require the assistance of a rheumatologist.

Antimetabolites—e.g., mycophenolate mofetil, methotrexate, azathioprine. This group of medications interferes with DNA synthesis, which is a crucial step in the proliferation of the cells that cause and regulate inflammation. T hese medications have been used successfully in the treatment of multiple systemic autoimmune conditions, and mycophenolate mofetil specifically is often used to prevent rejection after solid organ transplantation. Most commonly, side effects involve some type of gastrointestinal upset, but may also include liver toxicity, as well as a drop in white blood cell counts and one’s ability to fight off infections.

T-cell Inhibitors—e.g., cyclosporine, tacrolimus. These medications affect the production of certain chemical signals that allow T -cells (a type of white blood cell) to cause inflammation. Ultimately, they work by decreasing the amount of inflammation that can be caused by T -cells, the type of white blood cell that is thought to be responsible for most intraocular inflammation. T he most common side effects seen with these medications include kidney toxicity and high blood pressure, but may include more serious neurological effects as well.

Biologics—e.g., adalimumab (Humira®), infliximab (Remicade®), amongst many others. Biologics, so named because they have all been produced from living organisms or contain components of living organisms, have been an arena of significant development. By targeting certain cell types and molecules that are pivotal in the regulation of inflammation, these drugs are a valuable tool in the treatment of autoimmune and inflammatory diseases in general. Because the mechanism of each of these medications is slightly different from that of other drugs in this class, there is not necessarily a set of typical or expected side effects. H owever, it is important to note that many of these require regular injection at home (under the skin, as is the case with H umira), or intravenous administration at an infusion center. T hese medications, in general, may affect the body’s ability to fight off infection(s).

Ultimately, the treatment of uveitis may require multiple medications simultaneously (multiple different types of immunosuppressive medications), and may also require some systemic steroids or steroid injections in some cases where a multi-medication regimen is not sufficient in controlling one’s ocular inflammation.

Is this something that might go away on its own? How important is treatment?

Generally speaking, uveitis tends to affect both eyes (not necessarily symmetrically), and require chronic treatment. A s discussed previously, any amount of persistent, untreated intraocular inflammation has the potential to cause irreversible vision loss and structural damage to one’s eyes. T herefore, close follow up, at the very least, is warranted in most cases. Lifelong treatment may be required in some cases in which withdrawal of treatment results in recurrence of potentially damaging inflammation.

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