Managing Behcet's Disease
BY WILLIAM L. MILLER, OD, PHD, FAAO
Patients can remind us of the complex effect that systemic diseases can have on the eye. Recently I saw a television program about Behcet's disease, which brought back memories of one particular patient who taught me far more than any textbook could about the disease process and its effect on quality of life.
Signs and Symptoms
Behcet's disease mostly affects patients in the eastern Mediterranean and eastern Asian rim areas of the world. It's rare, with prevalence rates ranging from a low of 0.4/100,000 in the United States to a high of 370/100,000 in Turkey. A genetic predisposition exists for the disease, but it's sporadic.
Behcet's is an idiopathic, multisystem, chronic disease that waxes and wanes throughout a patient's lifetime. It affects patients in their 20s and 30s and diminishes their life expectancy, especially in males and in those who are diagnosed early in life with the disease.
First described nearly 70 years ago, the disease manifests with oral aphthae (98 percent of patients), genital ulcers (75 percent to 90 percent), skin lesions (75 percent to 87 percent) and ocular involvement (70 percent to 90 percent). This immune-mediated disease affects the venous system, resulting in episodes of vasculitis in the retinal vasculature. You may uncover other signs in a review of systems performed on a patient who has uveitis or retinal vasculitis.
A Behcet's patient may present with blurred vision, photophobia, ocular or periocular pain, lacrimation and hyperemia, all of which are nonspecific for ocular inflammation. The specific ocular signs affect the anterior and posterior segment with a worsened prognosis for the latter. Anterior segment inflammation may include iridocyclitis, iritis, scleritis, episcleritis or keratitis. The posterior segment may show signs of retinal vasculitis and may include occlusive disorders such as BRAO and BRVO. Other posterior segment abnormalities include vitritis, optic neuritis and papilledema. You may also observe retinal edema especially confined to the macular region.
Because the disease affects multiple organs, perform a careful review of systems in cases of recurrent bouts of anterior, intermediate, posterior and panuveitis. Additionally, the patient's care will take on a multidisciplinary approach involving optometry, ophthalmology, family practice, dentistry, obstetrics/gynecology and urology, to name a few. A coordinated treatment plan between each is essential to the management of this difficult condition.
Specific treatments in mild cases include topical steroids to suppress intraocular inflammation. Use topical steroids that penetrate the cornea (prednisolone acetate 1%) to treat anterior uveitis. Because of the multi-organ nature of Behcet's, you can administer steroids in an injectable form (subtenons in the case of ocular treatment), but oral and intravenous delivery of steroid medications may also subdue ocular and systemic inflammation. Colchicine can also control inflammation, working best for anterior inflammation while suggested as an adjunct to other medications for posterior segment inflammation. Antimetabolites such as Azathioprine and Methotrexate taken orally may also control inflammatory attacks.
Systemic administration of cyclosporine may control cases that are recalcitrant to the previously mentioned therapeutic approaches, especially in cases of severe ocular inflammation that are prone to dramatic visual impairment. Other medications currently showing promise for treating Behcet's disease include Thalidomide and immunomodulators and suppressors such as interferon a and Tracrolimus. Anti-cytokine agents are also being tested to more specifically target the inflammatory cascade.
Dr. Miller is on the faculty at the University of Houston College of Optometry. He is a member of the American Optometric Association and serves on its Journal Review Board. You can reach him at firstname.lastname@example.org.