Uveitis Incidence and Outlook: How Common Is It, and What Is the Prognosis?

“My eyes are red and painful,” “Light bothers me,” “My vision looks foggy”

These may be signs of a condition called uveitis.

Uveitis is by no means a rare disease.
Worldwide, 20 to 50 people per 100,000 develop it each year¹, and in Japan it accounts for 3.2% of new patients seen at specialty eye clinics², making it a relatively common inflammatory eye disease.

What Is Uveitis?

Uveitis is a disease in which inflammation occurs in the uvea, a tissue inside the eye.

The uvea consists of three parts, the iris, ciliary body, and choroid, and is rich in blood vessels, which makes it prone to inflammation. To use a camera as an analogy, it plays the role of an important component that adjusts the aperture and focus of the lens and regulates the light reaching the film.

When inflammation occurs, various symptoms appear, such as eye pain, redness, decreased vision, and sensitivity to light.

Incidence in the World and in Japan

Global Statistics

According to reliable epidemiological studies from around the world, the annual number of new uveitis patients is estimated at20 to 50 per 100,000 people^{3, 4}.

The prevalence (the number of people living with the disease at a given point in time) is100 to 150 per 100,000 people⁵, but higher rates have been reported in some regions, such as 700 per 100,000 in Sweden⁶and 580 per 100,000 in Thailand⁷.

One of the major characteristics of uveitis is the marked difference between regions and ethnic groups.

The Situation in Japan in Detail

In Japan, a2016 nationwide surveyconfirmed that new uveitis patients accounted for3.2%of all new patients at specialty eye clinics².

This shows that it is a relatively common disease in ophthalmology.

Ranking of Causes Identified in the 2016 Survey

1. Sarcoidosis (10.6%)
2. Vogt-Koyanagi-Harada disease (8.1%)
3. Herpetic iritis (6.5%)
4. Acute anterior uveitis (5.5%)
5. Behçet’s disease (4.2%)

Compared with earlier surveys, sarcoidosis remains the most frequent cause, while Behçet’s disease continues to decline.

Classification by Cause and Characteristics

The causes of uveitis fall into two broad groups.

Non-infectious uveitis

is a group of diseases caused mainly by abnormalities of the immune system⁸and accounts for the majority of uveitis.

Sarcoidosis and Vogt-Koyanagi-Harada disease, the most common causes in Japan, belong here.
This is a condition in which the body’s own immune system mistakenly attacks the eye tissue it is meant to protect.

Infectious uveitis

About14 to 20%⁹of uveitis patients have infectious uveitis.

Pathogens such as bacteria, viruses, fungi, and parasites enter the eye and cause inflammation.
It can be described as an outside invader running amok inside the body.

Uveitis: The Reality That the Cause Is Often Unknown

Surprisingly,in more than one in three uveitis patients, the underlying disease cannot be identified.¹⁰。

Even after detailed testing, unclassifiable cases account for more than one third.

Why does this happen? Uveitis is a very complex disease, and more than 30 different diseases can cause it. The reality is that even modern medicine has not been able to clarify every cause.

The large number of cases with unknown cause is itself one of the characteristics of uveitis.

Trends by Age and Sex

Differences by Sex

In surveys at university hospitals and general hospitals alike, a tendency formore female patients^11-13has been observed.

Women are known to develop uveitis more often, particularly the non-infectious forms related to autoimmunity.

Characteristics by Age

Overall, uveitis is seen most often in people in their40s and 50s, but the age of onset differs greatly depending on the disease^{2, 14}.

For example:

• Sarcoidosis: onset peaks in the 20s and after the age of 50, and after 50 it is especially common in women
• Behçet’s disease: most common in the 20s and 30s
• Chronic iridocyclitis in children: most common between the ages of 2 and 5

Age and sex alone cannot establish a diagnosis, but they are important clues for physicians in identifying the disease.

Uveitis: Regional Differences Exist Even Within Japan

Regional differences have been confirmed even within Japan.

HTLV-1-associated uveitisis seen in the Kyushu region (including the area around Fukuoka) atabout 3 to 5 times the frequencyof the national overall rate^15-17.

In addition,ocular toxoplasmosisalso occurs more frequently in the Kyushu region than the national average¹⁵.

Genetic factors, environmental factors, and lifestyle are thought to act in combination, but the detailed mechanisms remain a subject for future research.

Comparison of Incidence with Other Eye Diseases

Comparing the incidence of uveitis with that of other eye diseases makes its importance clearer.

Comparison with Retinal Detachment

Retinal detachment occurs at a rate of1 per 10,000 people per year(10 per 100,000 people per year)¹⁸.

In contrast, global statistics put new cases of uveitis at20 to 50 per 100,000 people per year⁴.

In other words,uveitis occurs about 2 to 5 times as often as retinal detachment.

Differences in Age Distribution

• Retinal detachment: a bimodal distribution with peaks in the 20s and in the 50s and 60s
• Uveitis: varies by disease, but overall is most common in the 40s and 50s

Retinal detachment is a relatively well-known eye disease, yet uveitis turns out to be far more frequent.

The Serious Impact of Uveitis on Vision

Uveitis is an important disease that accounts for10 to 15% of blindness worldwide.¹⁹

Without appropriate treatment, it can cause the following serious complications^{20, 21}:

• Glaucoma (damage to the optic nerve from elevated eye pressure)
• Cataract (clouding of the lens)
• Macular edema (swelling of the central part of the retina)
• Retinal detachment (a condition in which the retina peels away)

However, with early detection and appropriate treatment, it is also a disease in which vision can be preserved in many cases.

Symptoms to Watch For and When to See a Doctor

If you have symptoms such as the following, uveitis may be the cause:

• Red eyes
• Eye pain
• Sensitivity to light
• Decreased vision
• Vision that looks foggy
• Objects look distorted
• Seeing black spots or something like bugs (floaters)

These symptoms can also occur in other eye diseases.
However, because uveitis can seriously affect vision as it progresses, please see an eye doctor early.

In addition, uveitis is sometimes discovered even without symptoms, through referral from another department.
This is because uveitis can arise in association with systemic diseases.

Uveitis: Outlook and Prognosis

Many patients probably want to know what will happen to them in the future.

The prognosis of uveitis varies greatly depending on the underlying disease, the severity of inflammation, and the timing of treatment initiation.

Cases with a Favorable Course

• When an appropriate diagnosis and treatment are made early
• When the underlying disease is identified and effective treatment is available
• When the patient takes an active part in treatment

When these conditions are met, vision can be expected to be preserved or improved in many patients.

Cases That Require Caution

• When diagnosis or treatment is delayed
• When inflammation recurs repeatedly
• When serious complications occur

Even in such cases, there are now various treatment options.
Treatment tailored to each patient’s condition is possible, using corticosteroids, immunosuppressants, biologics, and other drugs.

The Importance of Long-Term Management

Uveitis is a disease that often follows a chronic course.
Like diabetes or hypertension, it may require long-term management.

With regular follow-up, recurrences of inflammation can be identified early and handled appropriately, which raises the likelihood of maintaining good visual function.

Medical Advances and New Treatments

Treatment of uveitis has advanced greatly in recent years.

In addition to conventional corticosteroids, new treatment options such as immunosuppressants and biologics have emerged. With these drugs, good results are now achievable even in cases that were previously difficult to treat.

Advances in diagnostic technology have also made more accurate and rapid diagnosis possible.
Imaging tests such as optical coherence tomography (OCT) and fluorescein angiography now allow the state of inflammation to be assessed in more detail.

The stages of treatment (eye drops, injections around the eye, oral medication, immunosuppressants, and biologics) and their respective side effects are explained in detail in a separate article.

A Message for Patients and Their Families

Even if you are diagnosed with uveitis, there is no need to give up.

With current medicine, many patients can preserve their vision and lead normal daily lives.
What matters, I believe, is to work with your doctor and stay patient and committed to treatment.

In fact, compared with 2001, when I became a resident, uveitis inflammation has become far easier to control.

Because uveitis can be associated with systemic disease, collaboration is often important not only with ophthalmology but also with internal medicine, rheumatology, and other departments.

(The above is general information. Please talk with your own doctor about your individual condition.)

Summary

Uveitis is by no means a rare disease.

The characteristics of its incidence can be summarized as follows^2-4:

• Worldwide, 20 to 50 per 100,000 people develop it each year
• In Japan, it accounts for 3.2% of new patients at specialty eye clinics
• Sarcoidosis is the most common cause (about 10.6%)
• More than one in three cases has an unknown cause
• Slightly more common in women
• Most often seen in people in their 40s and 50s
• Occurs 2 to 5 times as often as retinal detachment

With early diagnosis and appropriate treatment, this is a disease in which vision can be preserved in many cases. If you have symptoms that concern you, I recommend seeing an eye doctor without hesitation.

Uveitis is a complex and diverse disease, but thanks to advances in modern medicine, far better treatment outcomes can now be expected than before.

With accurate knowledge and appropriate medical care, it is entirely possible to live a fulfilling life while being monitored regularly.

References

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