New Behcet's management guidelines revealed.

Nine new recommendations for the management of Behcet's disease have been issued by the European League Against Rheumatism, based on a review of literature dating from 1966 through 2006.

Although the guidelines relating to the dermatologic, oral, ocular, and joint manifestations of Behcet's disease (BD) were mostly evidence-based, the recommendations on treating BD-associated vascular, neurologic, and gastrointestinal problems were "mainly based on observational studies, retrospective analyses, and clinical experience of the experts" who wrote the report (Ann. Rheum. Dis. 2008 Jan. 31 [doi: 10.1136 / ard.2007.080432]).

Dr. Yusuf Yazici, who was not on the task force, said in an interview the lack of randomized clinical trials in some areas of the disease can be explained by the fact that "these are rare manifestations and hard to recruit for." Dr. Yazici is the director of the Behcet's Syndrome Evaluation, Treatment and Research Center at the New York University Hospital for Joint Diseases. His father, Dr. Hasan Yazici, was one of the report's authors.

Dr. Yusuf Yazici added that there are "no good numbers" available to describe the prevalence of the disease in the United States. The disease affects anywhere between 1 and 6 people per 100,000, but "these are old numbers; no recent numbers are available," he said.

The recommendations are as follows:

* Regarding skin involvement, perceived severity should determine treatment. Topical steroids should be first-line treatment in genital and oral ulcers, while ache-like lesions can usually be treated with standard acne vulgaris treatments. In the literature, azathioprine was effective against resistant skin and mucosa lesions, while thalidomide was effective against oral and genital ulcers and papulopustular lesions.

* Treat posterior inflammatory eye disease with azathioprine and systemic corticosteroids. The authors cite a study (N. Engl. J. Med. 1990;322:281-5) where 2.5 mL/kg per day of azathioprine was efficacious in visual acuity and in halting disease progression.

* Severe eye involvement--greater than a 2-point drop in visual acuity on a 10/10 scale, or retinal disease--calls for a second immunosuppressive. "Cyclosporine A 2-5 mg/kg per day shows its effect rapidly and is, here, usually the treatment of choice," wrote the authors. Infliximab and interferon-[alpha] were also mentioned as candidates, though the latter is considered a second choice.

* For BD-associated acute deep vein thrombosis, corticosteroids, azathioprine, cyclophosphamide, or cyclosporine A is recommended. "The primary pathology leading to venous thrombosis in BD is the inflammation of the vessel wall," wrote the authors. "However, there are no RCTs addressing this issue." The same treatment is recommended for pulmonary and peripheral artery aneurysms.

* Pulmonary embolism is rare, so anticoagulants, antiplatelets, and fibrinolytic agents are not recommended. This is doubly true because of the chance of a coexisting pulmonary arterial aneurysm. Again, however, "controlled trials are needed."

* Immunosuppressants should be the first-line treatment over surgery in case of gastrointestinal ulcers, though no controlled trials exist to support one treatment specifically. "One study reported that azathioprine decreased reoperation rates and suggested that it should be used as maintenance therapy in patients who require surgery (Dis. Colon Rectum. 2000;43:692700)," wrote the authors.

* In most patients, arthritis can be managed with colchicine.

* For parenchymal involvement, "3-7 pulses of intravenous methylprednisone 1 gin/day is given during attacks, followed by maintenance oral corticosteroids which is tapered over 2-3 months." However, the authors caution that central nervous system involvement in BD is mostly based on anecdotal reports.

* Because it is neurotoxic, cyclosporine A should not be used in BD patients with CNS involvement unless intraocular inflammation makes it unavoidable.

"With proper management, remission is frequent in eye disease, skin-mucosa disease, and arthritis," according to Dr. Yazici, who added that although CNS disease and thrombotic manifestations pose considerable difficulties, "the disease usually gets better with time."

BY DENISE NAPOLI

Assistant Editor