Welcome to the Rare Classroom, a new series from Patient Worthy. Rare Classroom is designed for the curious reader who wants to get informed on some of the rarest, most mysterious diseases and conditions. There are thousands of rare diseases out there, but only a very small number of them have viable treatments and regularly make the news. This series is an opportunity to learn the basics about some of the diseases that almost no one hears much about or that we otherwise haven’t been able to report on very often.
Eyes front and ears open. Class is now in session.
The disease that we will be learning about today is:
Uveitis
What is Uveitis?
- Uveitis is the condition of inflammation affecting the uvea.
- The uvea is the pigmented layer situated between the outer fibrous layer (comprised of the sclera and cornea) and the inner retina.
- The uvea itself consists of three sub-layers:
- iris
- ciliary body
- choroid
- The condition is often described by which portion is affected, termed as anterior, intermediate, or posterior (front to back). Inflammation impacting all layers is dubbed panuveitis.
- The anterior type is the most common
- Generally, the eye is a well-protected environment, but at times the immune mechanisms can get overwhelmed
- The condition is considered an ophthalmic emergency that requires urgent examination and treatment
- Without prompt treatment, permanent vision loss may occur
- Rate of incidence is 1:4500
How Do You Get it?
- In many cases, uveitis appears on its own, and about half of anterior uveitis cases have no external cause
- People between age 20-60 are most likely to be affected
- Anterior uveitis is also associated with human leukocyte antigen B27 (HLA-B27)
- uveitis associated with HLA-B27 occurs more often in males
- Uveitis does occur as a symptom of a wide variety of conditions, such as:
- Autoimmune disorders, including but not limited to:
- Infections, including but not limited to:
- Zika fever
- Brucellosis
- Leptospirosis
- Lyme disease
- Systemic disorders, including but not limited to:
- White dot syndromes, including but not limited to:
- Serpiginous choroiditis
- Punctate inner choroiditis
- Acute zonal occult outer retinopathy
- Masquerade syndromes, including but not limited to:
- Retinitis pigmentosa
- Retinoblastoma
- Retinal detachment
What Are The Symptoms?
- Signs and symptoms vary depending on whether the patient has anterior, intermediate, or posterior uveitis.
- Anterior uveitis symptoms include:
- Eye redness
- Eye pain
- Synechia, in which the iris adheres to the lens or cornea
- Blurred vision
- Busacca nodules, which appear on the surface of the iris in some forms
- Irregular pupil
- Photophobia
- Hypopyon, in which immune cells appear in the anterior eye chamber (in severe cases)
- Intermediate uveitis symptoms include:
- Blurred vision
- Floaters, dark specks that appear in the field of vision
- Usually only one eye is affected
- Posterior uveitis symptoms include:
- Floaters
- Blurred vision
How Is It Treated?
- Treatment for uveitis should begin soon after diagnosis
- The most common therapy for the condition are glucocorticoid steroids.
- These may be administered orally or as topical eye drops.
- Corneal ulcers must be ruled out before treatment
- Topical cycloplegics may also be used
- Examples include homatropine and atropine
- In severe cases, a dose of subtenon triamcinolone may be used to reduce eye swelling.
- Methotrexate is also used in severe cases when other treatments don’t work.
- Metformin, which is used to treat diabetes, can inhibit the inflammation the causes uveitis.
- Infliximab has also been used successfully in experimental settings.
Where Can I Learn More???
- Check out our cornerstone on this disease here.
- Learn more about this condition from The Ocular Immunology and Uveitis Foundation.
