Behçet disease

Published on 18/03/2015 by admin

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Behçet disease

Sandra A. Kopp and Justin J. Green

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Behçet disease is a chronic inflammatory disorder leading to systemic vasculitis characterized by oral aphthae (at least three episodes in a 12-month period), plus two of the following: genital aphthae, cutaneous lesions (erythema nodosum, pustulosis, acneiform lesions, and pseudofolliculitis), uveitis or retinal vasculitis, or positive pathergy test. Arthritis, gastrointestinal, cardiac and neurologic manifestations may also occur. Many regimens effective for recurrent aphthous stomatitis are used to treat the aphthae of Behçet disease (see chapter on aphthous stomatitis).

Management strategy

In the absence of the multisystem disease, the severity and extent of the mucocutaneous manifestations direct the treatment strategy. First-line therapy for oral and genital aphthae is a high-potency topical corticosteroid in a gel or ointment formulation. Alternatively, intralesional corticosteroids (triamcinolone 5 mg/mL) can be used for major aphthae and severe minor aphthae. Other topical therapies accelerate healing or diminish the pain associated with oral aphthae. These include viscous lidocaine 2–5% applied directly to lesions, amlexanox 5% oral paste, sucralfate, tetracycline suspension and topical tacrolimus 0.1% ointment.

Colchicine 0.6 mg three times daily combined with topical corticosteroid therapy is efficacious in mucocutaneous disease. Dapsone 100–200 mg daily is also effective, but requires more frequent laboratory follow-up.

Those patients who fail the more conservative approaches or have severe mucocutaneous disease may require aggressive therapy. Thalidomide 100–300 mg daily (pediatric dose varies from 1 mg/kg/week to 1 mg/kg daily) is more effective than low-dose methotrexate 7.5–20 mg/week for severe disease. Prednisone taper begun at 1 mg/kg daily can be used for severe mucocutaneous flares, but rebound is a possible complication.

Systemic interferon (IFN)-α2a and anti-tumor necrosis factor (TNF)-α therapies may be best suited for those with severe mucocutaneous lesions and non-ocular systemic manifestations. Etanercept 50 mg weekly by subcutaneous injection, or infliximab 5 mg/kg in single or multiple intravenous infusions, have been shown to be effective in patients with recalcitrant disease and may be used as monotherapy or as an adjunct to conventional immunosuppressive therapy. Notably, infliximab has demonstrated rapid therapeutic effect, which may be useful in patients with vision-threatening posterior uveitis. These patients should be screened for latent tuberculosis infection prior to initiating anti-TNF therapies. Patients with certain extracutaneous signs (e.g., uveitis, aneurysms) may warrant combination therapy with prednisone and an immunosuppressive agent such as cyclosporine, azathioprine, chlorambucil or cyclophosphamide. Mucocutaneous disease alone rarely warrants this therapy; however, these agents have a beneficial effect on skin and mucous membrane lesions.

Specific investigations

First-line therapies

imageTopical/intralesional corticosteroid B
imageTopical tacrolimus A
imagePimecrolimus A
imageAmlexanox 5% paste A
imageSucralfate A
imageTetracycline suspension D
imageChlorhexidine gluconate D
imageColchicine A
imageZinc sulfate A

Second-line therapies

imageDapsone A
imageThalidomide A
imageMethotrexate E
imageSystemic corticosteroid B

Third-line therapies

imageIFN-α2a A
imageIsotretinoin E
imageCyclosporine A
imageAzathioprine A
imageCyclophosphamide E
imageChlorambucil D
imageMinocycline C
imagePenicillin B
imageProstaglandin E1 D
imageRebamipide A
imageEtanercept A
imageInfliximab D
imageAdsorption apheresis E
imageAzithromycin D
imageOral contraceptives E
imageNicotine patch D
imageMycophenolate sodium C
imageLenalidomide E
imageAlemtuzumab C

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Behçet disease

Sandra A. Kopp and Justin J. Green

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Behçet disease is a chronic inflammatory disorder leading to systemic vasculitis characterized by oral aphthae (at least three episodes in a 12-month period), plus two of the following: genital aphthae, cutaneous lesions (erythema nodosum, pustulosis, acneiform lesions, and pseudofolliculitis), uveitis or retinal vasculitis, or positive pathergy test. Arthritis, gastrointestinal, cardiac and neurologic manifestations may also occur. Many regimens effective for recurrent aphthous stomatitis are used to treat the aphthae of Behçet disease (see chapter on aphthous stomatitis).

Management strategy

In the absence of the multisystem disease, the severity and extent of the mucocutaneous manifestations direct the treatment strategy. First-line therapy for oral and genital aphthae is a high-potency topical corticosteroid in a gel or ointment formulation. Alternatively, intralesional corticosteroids (triamcinolone 5 mg/mL) can be used for major aphthae and severe minor aphthae. Other topical therapies accelerate healing or diminish the pain associated with oral aphthae. These include viscous lidocaine 2–5% applied directly to lesions, amlexanox 5% oral paste, sucralfate, tetracycline suspension and topical tacrolimus 0.1% ointment.

Colchicine 0.6 mg three times daily combined with topical corticosteroid therapy is efficacious in mucocutaneous disease. Dapsone 100–200 mg daily is also effective, but requires more frequent laboratory follow-up.

Those patients who fail the more conservative approaches or have severe mucocutaneous disease may require aggressive therapy. Thalidomide 100–300 mg daily (pediatric dose varies from 1 mg/kg/week to 1 mg/kg daily) is more effective than low-dose methotrexate 7.5–20 mg/week for severe disease. Prednisone taper begun at 1 mg/kg daily can be used for severe mucocutaneous flares, but rebound is a possible complication.

Systemic interferon (IFN)-α2a and anti-tumor necrosis factor (TNF)-α therapies may be best suited for those with severe mucocutaneous lesions and non-ocular systemic manifestations. Etanercept 50 mg weekly by subcutaneous injection, or infliximab 5 mg/kg in single or multiple intravenous infusions, have been shown to be effective in patients with recalcitrant disease and may be used as monotherapy or as an adjunct to conventional immunosuppressive therapy. Notably, infliximab has demonstrated rapid therapeutic effect, which may be useful in patients with vision-threatening posterior uveitis. These patients should be screened for latent tuberculosis infection prior to initiating anti-TNF therapies. Patients with certain extracutaneous signs (e.g., uveitis, aneurysms) may warrant combination therapy with prednisone and an immunosuppressive agent such as cyclosporine, azathioprine, chlorambucil or cyclophosphamide. Mucocutaneous disease alone rarely warrants this therapy; however, these agents have a beneficial effect on skin and mucous membrane lesions.

Specific investigations

First-line therapies

imageTopical/intralesional corticosteroid B
imageTopical tacrolimus A
imagePimecrolimus A
imageAmlexanox 5% paste A
imageSucralfate A
imageTetracycline suspension D
imageChlorhexidine gluconate D
imageColchicine A
imageZinc sulfate A

Pimecrolimus versus placebo in genital aphthous ulcers of Behçet’s disease: a randomized double-blind controlled trial.
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