Behcet’s Disease

What is Behcet's Disease?

Behçet's disease, also called Behçet's syndrome, is a rare autoinflammatory disease that causes inflammation of arteries and veins of all sizes. It is characterized by recurrent mouth and genital ulcers and by long-lasting, relapsing inflammation of the eye.^[1] It comes in flares and quiet periods, and it is most common along the old Silk Road, from the Mediterranean through the Middle East to East Asia.^{[2, 3]}

Common symptoms

Painful mouth ulcers are the most typical sign, and genital ulcers, skin lesions and eye inflammation are the other classic features.^[1] The disease can also involve the joints, blood vessels, nervous system and digestive tract, and symptoms differ a lot from person to person.^[2]

• Eye redness, pain or blurred vision from uveitis, which can threaten sight if untreated^[4]
• Painful red lumps (erythema nodosum) or acne-like skin lesions
• Swollen, painful joints
• Blood clots, mostly in veins, in up to half of patients^[5]
• Headache, confusion, weakness or stroke-like symptoms when the nervous system is involved^[6]
• Abdominal pain or diarrhea, and ongoing fatigue

Causes and risk factors

The cause is not known. Genetic, epigenetic, immune and infectious factors are all thought to contribute.^[2] The gene variant HLA-B*51 is the best-known genetic risk factor, but many people with the disease do not carry it, so other factors must also be involved.^[7]

How it is diagnosed and treated conventionally

There is no blood test or biopsy that confirms Behçet's disease, so doctors diagnose it from the pattern of symptoms and by ruling out other conditions.^[2] A skin prick (pathergy) test is part of some classification criteria, but it is positive much less often in regions where the disease is uncommon, and it can be positive in other conditions.^[8]

Treatment is tailored to which organs are involved. European guidelines recommend colchicine first for mouth ulcers, skin and joint disease, with apremilast or TNF inhibitors for hard-to-treat cases. Glucocorticoids and immunosuppressants are used for organ involvement, and TNF inhibitors are encouraged early for organ- or life-threatening disease.^[9] Care works best when a team of specialists shares it.^[4]

Why Behcet's Disease calls for a whole-person approach

Behçet's disease affects several body systems at once, and its course changes over time. Treatment decisions therefore depend on which organs are involved and how active the disease is.^[9]

Skin, mouth and joint symptoms do not usually cause permanent damage, but they can lower quality of life.^[4] That is where supportive care may have a role, alongside the specialist treatment that protects the eyes, blood vessels and brain.

Immune and inflammatory activity

Behçet's disease is driven by inflammation. Overactive neutrophils, a type of white blood cell, are thought to damage the blood vessel lining and promote clotting.^[5] This is why treatment relies mainly on drugs that calm the immune system.

Genes and environment

Inherited susceptibility, such as HLA-B*51, and environmental triggers such as infections are thought to act together.^{[2, 7]} Neither can be changed by complementary care.

Blood vessels and clotting

Clots in veins can occur in up to half of people with Behçet's disease, including in unusual places such as the brain or liver veins.^[5] Because the clots are driven by inflammation, they are treated mainly with immunosuppressants rather than blood thinners alone, which is a decision for the specialist.^[5]

Pain, ulcers and quality of life

Mouth and genital ulcers, joint pain and skin lesions are often what patients find hardest day to day, even when they are not dangerous.^[4] These symptoms are the main focus of any supportive care.

Acupuncture as supportive care in Behcet's Disease

What the research shows

Research on acupuncture for Behçet's disease is very limited, and there are no sham-controlled trials. A PubMed search found only a few small studies from China. In one randomized trial of 46 patients, those given acupuncture had a lower recurrence rate than those given drugs, but the report is brief and does not describe blinding.^[10] A study of 20 patients without a control group reported changes in an antibody measure and zinc levels after acupuncture, which does not show that symptoms improved.^[11]

For pain in general, a large pooled analysis of individual patient data found acupuncture reduced chronic musculoskeletal pain, osteoarthritis pain, headache and shoulder pain more than no acupuncture and, to a smaller degree, more than sham.^[12] Those results are not specific to Behçet's disease, so they may not apply to its joint or ulcer pain.

How acupuncture may work

How acupuncture might act in Behçet's disease is unknown. The small uncontrolled study above proposed that it changes immune function, but that idea has not been tested in a controlled trial.^[11] Pain relief is better studied than any effect on the immune system.^[12]

What treatment involves

Acupuncture uses fine, sterile, single-use needles placed at selected points, usually left in for about 20 to 40 minutes. A course of several visits is followed by a reassessment of whether it is helping.

Behçet's skin can overreact to needle injury. The pathergy reaction is a papule or pustule that forms one to two days after a sterile needle prick in some patients.^[8] Tell your practitioner about this and about your disease activity so the plan can be gentle, and report any new skin lesion after a session to your physician.

Minor reactions such as brief soreness, bleeding or bruising occur in about 9 in 100 patients, and serious events occur in about 1 in 10,000.^[13] Tell the practitioner first if you take blood thinners, have a bleeding disorder or a pacemaker, are pregnant, or take immunosuppressants that raise your risk of infection.

Ayurvedic medicine as supportive care in Behcet's Disease

The Ayurvedic view

Ayurveda has no classical name for Behçet's disease. Practitioners may interpret the recurring ulcers and inflammation as an excess of Pitta, the principle traditionally linked to heat and inflammation, with involvement of the blood. This is a traditional framework for choosing supportive care, not a medical diagnosis.

Therapies that may be used

Care is individualized and traditionally may include:

• Diet and daily routine, for example cooling, regular meals, avoiding very spicy or sour foods that irritate mouth ulcers, and steady sleep and stress-management habits
• Gentle oil massage
• Herbs, prescribed one person at a time by a qualified practitioner, with checks against your medicines

Panchakarma cleansing procedures are not appropriate during pregnancy, acute illness, frailty, or active flares involving the eyes, vessels or nervous system. They should not be considered while you are on strong immunosuppressants without your physician's agreement. Transdermal medication therapy, an herbal preparation applied to the skin, has no research in Behçet's disease.

What the research shows

There are no clinical trials or systematic reviews of Ayurvedic medicine for Behçet's disease. A PubMed search found none, so we cannot say whether it helps the disease. Research on turmeric (curcumin), an herb used in Ayurveda, is described in the herbal section below.

Metal contamination is a real risk with Ayurvedic products. About one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[14] Ayurvedic herbs can also interact with prescribed drugs, as other herbs do.^[15]

Herbal medicine as supportive care in Behcet's Disease

Herbs and formulas that have been studied

Most trials are of Chinese herbal decoctions, given alone or with conventional drugs. Gancao Xiexin decoction is discussed as a representative formula.^[16] Traditional Chinese medicine also describes Behçet's disease through its own pattern differentiation, such as damp-heat or yin deficiency with internal heat, and formulas are chosen to match those patterns.^[3] Turmeric (curcumin) has also been tested in a small trial.^[17]

What the research shows

The evidence is limited and low in quality. A meta-analysis of 8 trials found a higher response rate with herbal decoctions than with drug therapy, but the certainty was very low, methodological quality was low or very low, and the authors called for better trials.^[18] A review of 16 trials with 924 patients found decoctions beat control groups on most outcomes, and noted unsatisfactory study quality.^[16]

Another meta-analysis of 16 trials found that herbs added to drug therapy gave a better response rate than drugs alone, with low-certainty evidence. Oral ulcers, genital ulcers and eye inflammation were not improved more than with drugs alone.^[19] A placebo-controlled trial of 36 patients found that nanocurcumin for 8 weeks lowered disease activity and changed immune markers compared with placebo, but it was small and short.^[17] A review found only three studies of curcumin in Behçet's disease and called the evidence limited.^[20]

Safety and herb-drug interactions

Herbs can interact with the drugs used for Behçet's disease. Reported interactions include St John's wort lowering blood levels of ciclosporin and tacrolimus, which has led to organ rejection in transplant patients, and bleeding with warfarin and some herbs.^[15] Tell us about colchicine, azathioprine, ciclosporin, biologics, corticosteroids and any blood thinner before any herb is considered.

Liver problems, pregnancy and breastfeeding also call for caution. Herbal products can be contaminated, and Ayurvedic products have been found to contain lead, mercury and arsenic.^[14] Herbs should be prescribed individually and come from tested sources, and any herb should be stopped if you are scheduled for surgery or notice new symptoms.

Translating traditional medicine into modern biological language

This is interpretation, not equivalence. In Chinese medicine, Behçet's disease is described through patterns such as damp-heat and yin deficiency, and in Ayurveda recurring ulcers may be read as aggravated Pitta. Researchers instead study overactive neutrophils and other immune cells, inflammatory signals and damage to the blood vessel lining.^{[3, 5]}

The one proposed link in the research is that curcumin may calm inflammatory signaling and raise regulatory T cells, which restrain immune activity, but this comes from small studies.^{[17, 20]} No study has shown that a traditional pattern corresponds to a specific biological process in Behçet's disease.

How this care fits with your medical care

Behçet's disease needs ongoing care from a physician, usually a rheumatologist, with eye, neurology and vascular specialists when those organs are affected.^{[4, 9]} For a condition like this, we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care.

Do not stop or change colchicine, steroids, immunosuppressants or biologics without your prescriber. We do not diagnose or manage the disease. Please bring your diagnosis, recent test results, eye examination notes and a full list of medicines and supplements.

When to seek urgent medical care

Behçet's disease can cause sudden, serious problems in the eyes, blood vessels and brain.^{[5, 6]} Get medical help right away for:

• Sudden eye pain, redness, blurred vision or loss of vision
• Swelling, pain or redness in one leg, or sudden chest pain or shortness of breath, which may signal a clot
• Coughing up blood
• Severe headache, confusion, weakness, speech or vision changes, or seizures
• Severe or bloody abdominal pain or diarrhea
• Fever with a new skin lesion at a needle site

Call 911 or go to the nearest emergency department for any of these emergencies.