Putting Out Uveitis Fires

Case after case...

A six-year-old boy with a history of acute leukemia – and seemingly doing well after bone marrow transplantation and immunosuppressive therapy – developed blurred vision and uveitis in one eye and was referred to Wills Eye Hospital with suspicion of recurrent leukemia. Because the eye is an “immunologically privileged” site, intraocular inflammation in a child with a history of leukemia is recurrent leukemia until proven otherwise. The young patient’s family was understandably distraught.

A 25-year-old man came to the Wills Eye Uveitis Unit in the Retina Division for multiple recurrences of acute-onset pain, redness, and light sensitivity alternating between his eyes. At his examination, he was very surprised to be asked about low back pain, which he had suffered from for several years. He said, “My back is really stiff when I wake up, but then loosens up after an hour or two.” He was equally puzzled about the interest shown in his family history of inflammatory bowel disease, wondering what any of that had to do with his eyes.

A 32-year-old woman was referred to the Wills Eye Uveitis Unit for a severe, but unusual case of uveitis involving the front of one eye. It hadn’t responded to the typical regimen of steroid eye drops, and the iris was noted to be extremely thickened throughout, almost filling up the anterior chamber. She was promptly referred to the Uveitis Unit.

An 80-year-old accountant came to Wills from New York City because his eyes had become so red, irritated, and blurred that he could barely open them. He was no longer able to work, which he had continued into his 80s out of love for his job. He was seen in the Ocular Pemphigoid Clinic at Wills Eye, where the severe scarring of his conjunctiva and cornea in both eyes was evaluated by a team of uveitis, cornea, and oculoplastics specialists.

Day in, day out

The above cases are examples of complex ocular infectious and inflammatory eye diseases seen in the Retina Division’s Uveitis Unit at Wills Eye. As the unit’s director, I see about 500 new and 4,000 follow-up patients in my practice every year.

Uveitis – intraocular inflammation – is an uncommon, but potentially blinding collection of diseases that can be infectious or non-infectious, unilateral or bilateral, acute or chronic, and can affect any age group. Uveitis is described as the fifth or sixth (depending on the source) leading cause of blindness in the developed world because its complications include cataract, glaucoma, macular edema, and retinal scarring or detachment.

There are many different types and causes of uveitis; some are fairly easy to treat with a course of steroid drops or steroid injections in or around the eye, whereas others require antibiotic therapy, oral steroids, or immunosuppression. Often, uveitis is part of a disease affecting other parts of the body, and the ophthalmologist may be the first physician to make the connection. Proper diagnosis and prompt treatment can be not only vision-saving but, in some cases, even life-saving.

Working together, case after case

One of the advantages of seeing uveitis patients at Wills Eye is the potential for collaboration with other eye specialists. In some ways, a uveitis specialist is almost like a primary care physician, because so many uveitis patients suffer from diseases in other parts of their body.

In the cases listed above, the six-year-old boy with possible recurrent leukemia was seen with the ocular oncologists at Wills. His exam showed a viral infection in the eye (secondary to his immunosuppression after the bone marrow transplant), rather than recurrence of the leukemia, and he was successfully treated with antiviral therapy without having to undergo a painful bone marrow biopsy to evaluate for leukemia.

The 25-year-old man with recurrent episodes of uveitis was successfully treated with steroid eye drops, but also tested positive for a gene called HLA-B27. The gene is strongly associated with both recurrent uveitis and a type of arthritis called ankylosing spondylitis, which explained his low back pain. In collaboration with the rheumatologists at Thomas Jefferson University Hospital in Philadelphia, he was placed on a biologic immunosuppressant – adalimumab – that effectively controlled both his uveitis and his ankylosing spondylitis.

The 32-year-old woman with the uveitis and iris mass was referred to Wills Eye Ocular Oncology because her exam suggested a “masquerade syndrome,” one of a variety of diseases in which inflammation that looks like uveitis is actually caused by something else. In her case, a biopsy confirmed a rare type of lymphoma, and she was successfully treated in collaboration with Jefferson Oncology with combination chemotherapy known as R-CHOP – one of the very few cases in the world successfully treated in this way.

Finally, the 80-year-old accountant was diagnosed with a rare autoimmune eye disease, usually found in older patients, called ocular cicatricial pemphigoid. The Pemphigoid Clinic provided him a multidisciplinary team to evaluate and treat his corneal and conjunctival scarring and the abnormal eyelashes that were turned in and irritating his eyes. He was successfully treated with rituximab, a new therapy for pemphigoid, and was able to return to the accounting work he loved.