Interdisciplinary Autoimmune Summit
Interdisciplinary Autoimmune Summit
Source/Disclosures
Source:

Santos D. Exploring the Clinical Understanding of Behçet’s Disease (non-accredited). Presented at: Interdisciplinary Autoimmune Summit; July 10-12, 2020 (virtual meeting).

Disclosures: Santos reports that the presentation is supported by Amgen.
July 13, 2020
3 min read
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Behcet's disease remains largely unknown among physicians

Source/Disclosures
Source:

Santos D. Exploring the Clinical Understanding of Behçet’s Disease (non-accredited). Presented at: Interdisciplinary Autoimmune Summit; July 10-12, 2020 (virtual meeting).

Disclosures: Santos reports that the presentation is supported by Amgen.
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The rare and complex Behcet’s disease is often misdiagnosed or undiagnosed, leading to significant, but unnecessary, morbidity, according to a presenter at the 2020 Interdisciplinary Autoimmune Summit.

“It is not a commonly known condition,” Delfin Santos, MD, FACR, director of the Rochester Rheumatology Research Center in Rochester Hill, Michigan, said in his presentation. “A lot of physicians in many areas of medicine do not know about Behcet’s disease.”

The rare and complex Behcet’s disease is often misdiagnosed or undiagnosed, leading to significant, but unnecessary, morbidity, according to a presenter at the 2020 Interdisciplinary Autoimmune Summit. Source: Adobe Stock

Santos described Behcet’s disease as a chronic, multisystemic inflammatory disorder that is marked primarily by oral and genital ulcers, along with variable vessel vasculitis — meaning that it affects arteries and veins — that can bring manifestations to all systems of the body, from the skin to the eyes to neurological centers.

“Oral ulcers are the hallmark manifestation,” Santos said, noting that they can be major, minor or take a herpetiform. “Unfortunately, when patients first develop symptoms with oral and genital ulcers, they are frequently they are given a diagnosis of herpes. Psychologically, this is quite devastating to them when, in fact, they don’t have that.”

The oral and genital components of the disease can affect daily living, socializing and other associated quality of life (QOL) parameters. “This disease affects your ability to eat and talk,” Santos said. “Vaginal ulcers can lead to sexual dysfunction.”

In general, individuals with Behcet’s disease frequently report low self-esteem, negative body image, anxiety, fear and tension, according to Santos. He stressed that the chronic, debilitating nature of the condition makes it imperative that clinicians improve in the entire spectrum from diagnosis to treatment. “You have to plan your events around manifestations of this disease,” he said.

While the QOL parameters frequently top patient complaints, the small number of clinicians who are familiar with Behcet’s disease are concerned about its multisystemic nature. “The systems affected by this disease can be gastrointestinal, there are ocular lesions, the neurological system can be involved, there is swelling and pain, particularly in the proximal interphalangeal, or PIP, joints” he said. “The manifestations vary a little depending on what region of the world you live in.”

Behcet’s disease is primarily found along the Silk Road, but incidence can also be found in North America and northern Europe, according to Santos. He pointed out that gastrointestinal and neurological symptoms tend to be more prevalent among patients in the U.S. and northern Europe than those in Istanbul and other areas in the Silk Road region, according to findings from key data sets.

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Other findings from regional studies have shown that Behcet’s disease is more prevalent among women than men in both the U.S. and in the Silk Road regions, while disease duration is longer in the U.S. “It can burn out over time,” Santos added.

In addition, U.S. patients reported higher disease activity than those in Turkey, according to Santos.

Turning to the differential diagnosis, Santos highlighted that disease duration factors in this discussion. “The average time to diagnosis is 5.3 years,” he said.

The first issue to consider in diagnosis is that while oral or genital lesions are generally the first symptom of the disease, they may occur intermittently. Moreover, as long as 8 years may pass until a second manifestation occurs.

Another consideration is that Behcet’s disease may present as a number of diseases in the rheumatic and autoinflammatory spectrum, including Crohn’s disease. “Crohn’s can have all the manifestations of Behcet’s disease, including gastrointestinal, oral, ocular and skin,” he said.

Clinicians must rely on the International Study Group criteria for Behcet’s disease, which was developed in 1990. “Everyone has to have mouth sores three times within a 12-month period,” Santos said. To receive a diagnosis, a patient must also have at least one other criteria, including genital ulcers, eye lesions, skin lesions or pathergy.

Santos is heartened by last year’s approval of apremilast (Otezla, Amgen) for oral ulcers associated with Behcet’s disease, the first approval for the condition. “Much needs to be learned and investigated,” Santos said.