In a bid to improve awareness and care of a relatively rare disorder that inflames blood vessels throughout the body and damages organs, especially the eyes, a team of researchers led by a Johns Hopkins ophthalmologist has analyzed and published detailed information about 132 patients diagnosed and treated over 25 years in Saudi Arabia, where the disorder is not at all rare.
Among other findings, the researchers report that a long-standing combination of immunosuppressive drugs, such as steroids and surgery to offset blindness and other serious complications, remains the best way so far to treat Behcet’s disease.
The precise cause of the disorder, which also causes skin, genital, brain and other complications, is not known, but Behcet’s is believed to be an autoimmune disease with both inherited and environmental triggers, the researchers say.
Named after the Turkish dermatologist who first described the ailment in 1937, Behcet’s disease is marked by a constellation of sores, swelling and symptoms in the eyes, mouth, genitals, gastrointestinal tract and nervous system. Though rare globally, it is more widely diagnosed in the so-called Silk Road regions of Asia and the Middle East, and it affects an estimated one in 10,000 people in Saudi Arabia.
The current case series study published in the January issue of the American Journal of Ophthalmology was led by Fernando Arevalo, M.D., professor of ophthalmology at the Johns Hopkins University School of Medicine, who is currently based at Saudi Arabia’s King Khaled Eye Specialist Hospital, which has been affiliated with Johns Hopkins Medicine since 2010.
Because little is known about how to treat patients with Behcet’s worldwide, particularly in countries like the United States, where the disease is rarest, Arevalo and his colleagues sought out cases where information was likely to be plentiful. The King Khaled Eye Specialist Hospital is a national referral center for those with the disorder, due to the commonplace impact of the disease on vision. The case review results, described in the January issue of the American Journal of Ophthalmology, offers details of the eye problems that these patients were most likely to experience. Topping the list, Arevalo and team say, was panuveitis, an inflammation marked by redness and swelling that encompasses the entire eyes of nearly 90 percent of those eventually diagnosed with Behcet’s. About three-quarters of patients experienced this condition in both eyes, and it significantly diminished their visual acuity. The average eyesight of those affected was 20/125, even with corrected vision.
Other patients had symptoms that included inflammation of the retina and its blood vessels, swelling of the macula — the portion of the eye responsible for high-acuity vision — glaucoma and cataracts. One of the most serious complications of Behcet’s is optic nerve atrophy, a condition that can lead to irreversible blindness.
Though diagnosing Behcet’s can be tricky, the researchers report that given a definitive diagnosis, the disease often responds well to treatment with a combination of corticosteroids and other immunosuppressive drugs. About three-quarters of patients tended to improve substantially with drug treatment alone.
Some 26 percent of the patients whose cases were reviewed needed eye surgery to control symptoms or complications, with 71 percent of those procedures done to remove cataracts or clouded lenses. After combination drug and surgical therapy, about one-half of patients had 20/50 corrected vision or better, the authors say.
Knowing the characteristics of the disease and the best ways to treat it are crucial for ophthalmologists and other doctors who may not suspect its presence.
Arevalo notes that during the 25 years the case review covers, from 1986 to 2011, newer medications, including monoclonal antibodies and other so-called biologic pharmaceuticals, weren’t widely used to treat this condition. However, these medications are showing promise, and he and his colleagues plan to compare the newer drugs to older immunosuppressive therapies. In fact, in their series, they found that only the combination of prednisone, cyclosporine and infliximab caused a significant improvement in vision. The authors’ findings support recent reports that suggest that infliximab, a biologic drug, reduces the frequency of ocular attacks and improves visual acuity in patients with Behcet’s disease-related uveitis.
Other researchers who contributed to this study are Hassan A. Al Dhibi, Mohanna Yousef Al Jindan, Ammar M. Al-Mahmood, Yahya A. Al-Zahrani and Hassan A. Al Dhibi of King Khaled Eye Specialist Hospital, Saudi Arabia; Nasser Abdulmohsen Al Sabaani of the College of Medicine, King Khalid University, Saudi Arabia; Andres F. Lasave of the Clinica Privada de Ojos, Argentina; and the Kkesh Uveitis Survey Study Group.