Behcet’s Disease Diagnosis: Understanding the Path to Identification

Diagnosing Behcet’s disease can be a complex journey. There isn’t a single definitive test to confirm its presence, making the process reliant on a thorough evaluation of your signs and symptoms. A key indicator that doctors look for is recurrent mouth sores, as these are experienced by nearly everyone with Behcet’s. In fact, the appearance of mouth sores at least three times within a year is often a crucial factor in considering a Behcet’s disease diagnosis.

Diagnostic Criteria for Behcet’s Disease

Beyond recurrent mouth sores, diagnosing Behcet’s disease requires the presence of at least two additional signs. These signs can vary from person to person, but commonly include:

  • Recurring Genital Sores: Painful sores that appear on the genitals.
  • Eye Inflammation (Uveitis): Inflammation affecting parts of the eye, which can cause pain, redness, and vision problems.
  • Skin Sores: Various types of skin lesions or bumps that can occur on different parts of the body.

It’s important to note that these symptoms can also be associated with other conditions, making a careful and comprehensive assessment essential for an accurate Behçet’s disease diagnosis.

Diagnostic Tests Used in Evaluating Behcet’s Disease

While no test directly diagnoses Behcet’s disease, certain tests play a vital role in ruling out other conditions and supporting the overall diagnostic picture. These may include:

  • Blood Tests and Laboratory Analysis: These tests are primarily used to exclude other diseases that might mimic Behcet’s symptoms. They can help doctors differentiate Behcet’s from infections, autoimmune disorders, or other inflammatory conditions.

  • Pathergy Test: This unique skin sensitivity test is often used in Behcet’s diagnosis. The procedure involves a doctor inserting a sterile needle, typically into the forearm skin. The area is then examined after one to two days. A positive Pathergy test is indicated by the formation of a small, red bump at the needle insertion site. This reaction suggests that the immune system is overreacting to minor trauma, a characteristic often seen in Behcet’s disease. It’s important to understand that the Pathergy test is not always positive in individuals with Behcet’s, and a negative result does not exclude the diagnosis.

Treatment Approaches for Behcet’s Disease

Currently, there is no cure for Behcet’s disease. Treatment strategies are focused on managing symptoms, reducing the frequency and severity of flares, and improving the patient’s quality of life. Treatment plans are highly individualized and depend on the specific symptoms and their severity.

For individuals with mild Behcet’s disease, treatment may primarily involve medications to manage pain and inflammation during flares. In these cases, medication may not be necessary between flare-ups.

However, more severe cases of Behcet’s disease require a more comprehensive approach. Doctors may prescribe medications to control the disease’s activity throughout the body, in addition to treatments for managing acute flares.

Targeted Treatments for Specific Behcet’s Symptoms

To address the discomfort and inflammation associated with flares, several localized treatments can be effective:

  • Topical Skin Creams, Gels, and Ointments: Corticosteroid-based topical medications are directly applied to skin and genital sores to reduce inflammation and alleviate pain.
  • Therapeutic Mouth Rinses: Special mouthwashes containing corticosteroids or other anti-inflammatory agents can significantly reduce the pain and discomfort of mouth sores.
  • Anti-inflammatory Eyedrops: For mild eye inflammation, eyedrops containing corticosteroids or other anti-inflammatory medications can effectively relieve pain and redness.

Systemic Treatments for Behcet’s Disease Management

When topical treatments are insufficient, or for more widespread disease, systemic medications are often necessary.

  • Colchicine: For recurring oral and genital sores, colchicine (Colcrys, Mitigare) may be prescribed. This medication can also help improve joint swelling associated with Behcet’s.

For moderate to severe Behcet’s disease, treatments aim to control the disease activity and prevent long-term damage between flares. These may include:

  • Corticosteroids: Medications like prednisone are potent anti-inflammatories that effectively reduce the inflammation caused by Behcet’s disease. They are often prescribed in combination with other immunosuppressant medications. It’s important to be aware of potential side effects of corticosteroids, including weight gain, persistent heartburn, high blood pressure, and osteoporosis.

  • Immunosuppressant Medications: These drugs work by suppressing the immune system’s overactivity, which is central to Behcet’s disease. Common examples include azathioprine (Azasan, Imuran), cyclosporine (Gengraf, Neoral, Sandimmune), and cyclophosphamide. While effective, these medications can increase the risk of infections and may have other side effects such as liver and kidney problems, low blood cell counts, and high blood pressure.

  • Immune System Modulators: Interferon alfa-2b (Intron A) is a medication that helps regulate immune system activity to control inflammation. It can be used alone or in combination with other drugs to manage skin sores, joint pain, and eye inflammation in Behcet’s patients. Flu-like symptoms, such as muscle pain and fatigue, are potential side effects.

  • Tumor Necrosis Factor (TNF) Blockers: For more severe or treatment-resistant Behcet’s symptoms, particularly those affecting the eyes and major organs, medications that block TNF can be highly effective. Examples include infliximab (Remicade) and adalimumab (Humira). Possible side effects include headache, skin rash, and an increased susceptibility to infections.

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Coping Strategies and Support Systems for Behcet’s Disease

Living with the unpredictable nature of Behcet’s disease can be emotionally and physically challenging. Adopting self-care strategies and seeking support are crucial aspects of managing the condition effectively. Consider these approaches:

  • Prioritize Rest During Flares: When symptoms flare up, allow yourself adequate rest. Adjust your schedule to accommodate your need for rest and minimize stress, which can exacerbate symptoms.
  • Maintain Activity When Possible: During periods of remission, engage in moderate exercise like walking or swimming. Regular physical activity can improve mood, strengthen your body, and maintain joint flexibility.
  • Connect with Support Networks: Behcet’s disease is relatively rare, which can sometimes lead to feelings of isolation. Seek out support groups in your area or online communities like the American Behcet’s Disease Association’s message boards and chat rooms to connect with others who understand your experience.

Preparing for Your Doctor’s Appointment

Your initial consultation will likely be with your primary care physician, who may then refer you to a rheumatologist (a specialist in arthritis and rheumatic diseases). Depending on your specific symptoms, you may also be referred to specialists such as ophthalmologists (for eye problems), gynecologists or urologists (for genital sores), dermatologists (for skin issues), gastroenterologists (for digestive problems), or neurologists (for neurological symptoms).

To make the most of your appointment, consider these steps:

What You Can Do Beforehand

  • Symptom Journal: Keep a detailed record of your symptoms, noting when they started, their severity, and any triggers you identify.
  • Personal Information Summary: Prepare a brief overview of key personal information, including major stressors or recent life changes that might be relevant.
  • Medication List: Compile a list of all medications, vitamins, and supplements you are currently taking, including dosages.
  • Questions for Your Doctor: Write down a list of questions to ensure all your concerns are addressed during the appointment.

Bringing a family member or friend can be helpful to assist with remembering information and providing support.

For Behcet’s disease, some key questions to ask your doctor include:

  • What do you believe is the underlying cause of my symptoms?
  • What specific tests are necessary? Do these tests require any special preparation?
  • Is Behcet’s disease a temporary or chronic condition?
  • What are the available treatment options, and what treatment approach do you recommend for me?
  • I have other pre-existing medical conditions. How can Behcet’s disease and these conditions be managed together effectively?
  • Do you have any brochures, printed materials, or reliable websites that you would recommend for further information on Behcet’s disease?

What to Expect From Your Doctor

Your doctor will ask you detailed questions to gain a comprehensive understanding of your symptoms and medical history. Be prepared to answer questions such as:

  • Are your symptoms constant, or do they fluctuate and come and go?
  • Are there any factors or activities that seem to improve your symptoms?
  • Are there any factors that seem to worsen your symptoms?
  • Is there a family history of similar illnesses or autoimmune conditions?

By Mayo Clinic Staff

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