Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

mucocutaneous ulcers

1st line – topical triamcinolone paste

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ACUTE
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mucocutaneous ulcers
1st line – 

topical triamcinolone paste

For mild symptoms, treatment with a topical corticosteroid (i.e., triamcinolone oral paste) as needed is usually sufficient.[19]

The most severe manifestation present in the patient should determine the treatment choices pursued, regardless of whether less severe manifestations are also present.

Primary options

triamcinolone topical: (0.1%) apply to the affected area(s) up to three times daily

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2nd line – colchicine or oral corticosteroid or immunosuppressive therapy

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ACUTE
|
mucocutaneous ulcers
2nd line – 

colchicine or oral corticosteroid or immunosuppressive therapy

Lesions resistant to local measures may require systemic treatment with colchicine, an oral corticosteroid, or another immunosuppressive agent (e.g., azathioprine, hydroxychloroquine, thalidomide).

Follow-up of participants in a placebo-controlled clinical trial confirmed that colchicine did not reduce the need for subsequent immunosuppressive drugs.[24]

In a 12-week phase 3 trial of patients with Behcet's syndrome, apremilast significantly improved measures of disease-related quality of life and reduced the number of oral ulcers compared with placebo.[25] Adverse events associated with apremilast included diarrhoea, nausea, and headache.[25]

The most severe manifestation present should determine the treatment choices pursued, regardless of whether less severe manifestations are also present.

Treatment duration is usually 1-2 years for active disease. Corticosteroids and colchicine can be used as needed. Azathioprine may need 6 months to achieve maximum efficacy.

Primary options

colchicine: 0.6 mg orally two to three times daily

OR

prednisolone: 1 mg/kg/day orally

OR

azathioprine: 2.5 mg/kg/day orally

OR

hydroxychloroquine: 200 mg orally twice daily

OR

thalidomide: 50-200 mg orally once daily

OR

apremilast: 30 mg orally twice daily

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3rd line – tumour necrosis factor (TNF)-alpha inhibitor

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ACUTE
|
mucocutaneous ulcers
3rd line – 

tumour necrosis factor (TNF)-alpha inhibitor

Monotherapy with infliximab, adalimumab, or etanercept may be helpful in mild Behcet's syndrome resistant to corticosteroids and oral immunosuppressants, but the potential toxicities of these agents should be weighed against the individual patient's clinical condition.

The most severe manifestation present in the patient should determine the treatment choices pursued, regardless of whether less severe manifestations are also present.

Treatment duration is usually 1-2 years for active disease.

Primary options

infliximab: consult specialist for guidance on dose

OR

adalimumab: consult specialist for guidance on dose

OR

etanercept: consult specialist for guidance on dose

eye involvement

1st line – prednisolone plus azathioprine

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ACUTE
|
eye involvement
|
mild to moderate
1st line – 

prednisolone plus azathioprine

Severity is judged by an ophthalmologist, and treatment must be carried out in close collaboration with an ophthalmologist.[19]

Azathioprine plus a corticosteroid is a commonly used drug regimen. Corticosteroids should never be used alone but should always be given with a systemic immunosuppressant in patients with posterior uveitis.[19]

The corticosteroid can be tapered in many patients after active disease has been controlled, whereas the immunosuppressants are generally continued for at least 1 to 2 years.

The most severe manifestation present in the patient should determine the treatment choices pursued, regardless of whether less severe manifestations are also present.

Treatment duration is usually 1-2 years for active disease. Azathioprine may need 6 months to achieve maximum efficacy.

Primary options

prednisolone: 1 mg/kg/day orally

and

azathioprine: 2.5 mg/kg/day orally

1st line – prednisolone plus immunosuppressant therapy

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ACUTE
|
eye involvement
|
severe or resistant
1st line – 

prednisolone plus immunosuppressant therapy

If eye manifestations are judged severe by the ophthalmologist, or if they do not resolve with azathioprine plus a corticosteroid, a tumour necrosis factor-alpha inhibitor (e.g., infliximab or adalimumab) or ciclosporin may be added to the regimen.[26] Ciclosporin has the advantage of having a more rapid action than azathioprine (6 months vs. 3 months). Treatment requires close collaboration with an ophthalmologist.[19]

The most severe manifestation present in the patient should determine the treatment choices pursued, regardless of whether less severe manifestations are also present.

Treatment duration is usually 1-2 years for active disease. Azathioprine may need 6 months to achieve maximum efficacy.

Primary options

prednisolone: 1 mg/kg/day orally

-- AND --

azathioprine: 2.5 mg/kg/day orally

-- AND --

infliximab: consult specialist for guidance on dose

or

adalimumab: consult specialist for guidance on dose

or

ciclosporin: 2.5 to 5 mg/kg/day orally

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gastrointestinal involvement

1st line – prednisolone plus immunosuppressant therapy

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ACUTE
|
gastrointestinal involvement
1st line – 

prednisolone plus immunosuppressant therapy

Corticosteroids and azathioprine are used alone, or in combination with a tumour necrosis factor (TNF)-alpha inhibitor. Infliximab is preferred over other TNF-alpha inhibitors, as there are fewer data with adalimumab, and etanercept does not seem to work as well.

The most severe manifestation present in the patient should determine the treatment choices pursued, regardless of whether less severe manifestations are also present.

Treatment duration is usually 2-5 years for active disease. It may be needed lifelong. Azathioprine may need 6 months to achieve maximum efficacy.

Primary options

prednisolone: 1 mg/kg/day orally

and

azathioprine: 2.5 mg/kg/day orally

OR

prednisolone: 1 mg/kg/day orally

and

azathioprine: 2.5 mg/kg/day orally

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and

infliximab: consult specialist for guidance on dose

central nervous system involvement

1st line – prednisolone plus immunosuppressant therapy

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ACUTE
|
central nervous system involvement
|
parenchymal disease
1st line – 

prednisolone plus immunosuppressant therapy

Treatment of acute parenchymal disease is with high-dose corticosteroids, which are gradually tapered and given in combination with an immunosuppressive agent (e.g., azathioprine or cyclophosphamide). Azathioprine may need 6 months to achieve maximum efficacy.

A tumour necrosis factor (TNF)-alpha inhibitor (e.g., infliximab) is an alternative or additional first-line agent in patients with severe involvement, disease that is refractory despite standard treatment, or chronic progressive parenchymal disease.[19] This recommendation is based on the results of uncontrolled studies that suggest a benefit from treatment with a TNF-alpha inhibitor; either in combination with other immunosuppressants, or as monotherapy, following unsuccessful treatment with standard agents.[27][28][29]

Primary options

prednisolone: 1 mg/kg/day orally

-- AND --

azathioprine: 2.5 mg/kg/day orally

or

cyclophosphamide: 2.5 mg/kg/day orally; or 1000 mg/square metre of body surface area intravenously once monthly

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-- AND --

infliximab: consult specialist for guidance on dose

OR

infliximab: consult specialist for guidance on dose

1st line – prednisolone ± azathioprine

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ACUTE
|
central nervous system involvement
|
acute cerebral venous thrombosis
1st line – 

prednisolone ± azathioprine

Treatment for complicated venous thrombotic events such as acute cerebral venous thrombosis in Behcet's syndrome is with a high-dose corticosteroid for rapid remission. Another immunosuppressant, such as azathioprine, may be added if necessary.[19] Azathioprine may need 6 months to achieve maximum efficacy.

Primary options

prednisolone: 1 mg/kg/day orally

OR

prednisolone: 1 mg/kg/day orally

and

azathioprine: 2.5 mg/kg/day orally

Consider – anticoagulation

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ACUTE
|
central nervous system involvement
|
acute cerebral venous thrombosis
Consider – 

anticoagulation

Additional treatment recommended for SOME patients in selected patient group

Anticoagulants may be used in some patients, provided that the overall risk of bleeding is low and that co-existent pulmonary artery aneurysms are ruled out. However, anticoagulation is not part of the standard treatment approach. Consult specialist for choice of anticoagulation regimen.

major vascular involvement

1st line – prednisolone plus immunosuppressant therapy

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ACUTE
|
major vascular involvement
|
systemic venous thrombosis
1st line – 

prednisolone plus immunosuppressant therapy

Venous thrombotic events are managed in Behcet's syndrome by controlling systemic inflammation with corticosteroid treatment in combination with immunosuppressants such as azathioprine, cyclophosphamide, or ciclosporin.[19] Cyclophosphamide may be reserved for patients with extensive thrombosis of larger veins such as the vena cava because of potential adverse events.[19] Azathioprine may need 6 months to achieve maximum efficacy.

Tumour necrosis factor-alpha inhibitors in combination with corticosteroids can be considered for patients with refractory venous thrombosis.[19] One retrospective study of 70 patients with Behcet's syndrome-associated venous thrombosis reported that adalimumab-based treatments were more effective than azathioprine, ciclosporin, cyclophosphamide, and methotrexate for inducing rapid resolution of venous thrombosis.[31]

Primary options

prednisolone: 1 mg/kg/day orally

-- AND --

azathioprine: 2.5 mg/kg/day orally

or

cyclophosphamide: 2.5 mg/kg/day orally; or 1000 mg/square metre of body surface area intravenously once monthly

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or

ciclosporin: 2.5 to 5 mg/kg/day orally

or

adalimumab: consult specialist for guidance on dose

Consider – anticoagulation

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ACUTE
|
major vascular involvement
|
systemic venous thrombosis
Consider – 

anticoagulation

Additional treatment recommended for SOME patients in selected patient group

Anticoagulants may be used in some patients, provided that the overall risk of bleeding is low and that co-existent pulmonary artery aneurysms are ruled out. However, anticoagulation is not part of the standard treatment approach. Consult specialist for choice of anticoagulation regimen.

1st line – corticosteroid plus immunosuppressant therapy

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ACUTE
|
major vascular involvement
|
arterial involvement
1st line – 

corticosteroid plus immunosuppressant therapy

Pulmonary arterial aneurysms and thromboses are treated with high-dose corticosteroids and cyclophosphamide.[19]

Peripheral arterial aneurysms are treated with corticosteroids and cyclophosphamide. Small arterial aneurysms may be successfully managed with medical therapy alone.[19]

Primary options

methylprednisolone: consult specialist for guidance on dose

and

prednisolone: 1 mg/kg/day orally (after methylprednisolone)

and

cyclophosphamide: 1000 mg/square metre of body surface area intravenously once monthly

Consider – embolisation, stenting, or surgery

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ACUTE
|
major vascular involvement
|
arterial involvement
Consider – 

embolisation, stenting, or surgery

Additional treatment recommended for SOME patients in selected patient group

Embolisation of pulmonary arterial aneurysms may be necessary if there is a high risk of major bleeding. Open surgery should only be considered in life-threatening situations.[19]

In most patients with peripheral arterial aneurysms, surgery or stenting is required in addition to medical therapy.[19]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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