Treatment of Behcet’s Disease


Apremilast (Otezla), an oral selective phosphodiesterase 4 (PDE4) inhibitor, is the first and only approved treatment by the US Food and Drug Administration (FDA) for oral ulcers associated with Behcet's Disease. Apremilast was approved as a 30 mg twice-daily therapy for adult patients in the United States. There are currently about 5 in 100,000 US residents affected by Behcet's Disease. Multiple systems can be affected by the disease, but it's often characterized by recurrent oral ulcers accompanied by lesions in other organ systems.

Otezla's approval was based on the efficacy and safety results of a randomized, double-blind, placebo-controlled phase 3 RELIEF study, in which 207 previously-treated adult patients with Behcet’s Disease and active oral ulcers were treated with either therapy or placebo.

Treatment of Behcet's Disease typically focuses on reducing discomfort and preventing serious complications. Although Behcet's is a chronic disease, patients may have periods of time when symptoms disappear temporarily (remission). The severity of the disease varies from one patient to another. Some patients may experience milder symptoms, while others may develop complications affecting various organ systems. It is important to treat the manifestations of the disease accordingly.

Behcet’s Disease affects different parts of the body, therefore; it is likely patients will have different doctors. It will be helpful to have a primary care physician in order to coordinate treatment and monitor care. Communication among various physicians is very important in order to achieve optimal care.

Rheumatologists (doctors specializing in arthritis and other autoimmune and inflammatory disorders) are the most knowledgeable about the disease and should be the main physicians involved in the care and treatment of Behcet's Disease patients.

Other doctors involved in coordinated care may include:

Specialized DoctorWhat is treated
DermatologistTreats skin related problems
GynecologistTreats the health of the female reproductive system
UrologistTreats male and female urinary system and male reproductive organs
GastroenterologistManages diseases of the gastrointestinal tract and liver
PulmonologistDeals with diseases involving the respiratory tract
Otorhinolaryngologist (ENT)Treats ears, nose and throat
OphthalmologistTreats eye disorders
NephrologistSpecializes in kidney care
HematologistTreats disorders of the blood
NeurologistTreats disorders of the nervous system
Psychiatrist or PsychologistTreats mental health disorders
DentistTreats oral ulcers and other oral problems
Internist or Family Practice PhysiciansPrimary care doctors
Pain Management SpecialistHelp treat chronic pain

Patients can control symptoms of Behcet's Disease with proper medication, rest, exercise, and a healthy lifestyle. The goal of treatment is to reduce discomfort and prevent serious complications. It is likely that a combination of medicines will be used in the treatment of Behcet's Disease, depending on symptoms. Patients must provide their doctors with a list of all medications they are currently taking.

Drugs Used in the Treatment of Behcet's Disease

Topical medication is applied directly to the ulcers and skin lesions in order to relieve pain and discomfort. Rinses, gels or oils may be prescribed. Creams are used to treat skin and genital lesions. The medication may contain corticosteroids, other anti-inflammatory drugs, or an anesthetic to relieve pain. Topical glucocorticoids used to treat symptoms of Behcet's Disease may include:

  • Triamcinalone acetonide used to treat oral ulcers
  • Betamethasone ointment used to treat genital ulcers
  • Betamethasone drops used for anterior uveitis and retinal vasculitis
  • Dexamethasone drops used as therapy for retinal vasculitis

Please note: Most of these drugs have not received FDA approval for the treatment of Behcet's Disease but may help reduce the symptoms associated with inflammatory diseases.

TNF inhibitors (anti tumor necrosis factor drugs) are a class of drugs used in the United States for more than 15 years and used worldwide to treat inflammatory conditions. These biologic agents have the capacity to reduce inflammation and slow disease progression by targeting TNF, an inflammation causing substance. TNF inhibitors are used in many diseases including rheumatoid arthritis, juvenile rheumatoid arthritis, psoriasis, psoriatic arthritis, ankylosing spondylitis, ulcerative colitis and Crohn's disease. Some of these drugs include:

  • Enbrel (etanercep)
  • Remicade (infliximab)
  • Humira (adalimumab)
  • Cimzia (certolizumab)
  • Simponi (golimumab)
  • Azasan, Imuran (azathioprine)
  • Neoral, Sandimmune, and Gengraf (cyclosporine)
  • Cytoxan, Neosar (cyclophosphamide)
  • Prednisone is used as therapy for gastrointestinal lesions, acute meningoencephalitis, chronic central nervous system lesions, arthritis, erythema nodosum, anterior uveitis and retinal vasculitis
  • Methylprednisolone is used in the treatment of gastrointestinal lesions, acute meningoencephalitis, chronic, progressive central nervous system lesions, arteritis, retinal vasculitis and venous thrombosis
  • Otezla (apremilast) is the first and only FDA approved treatment for oral ulcers of Behcet's Disease in adult patients in the US
  • Methotrexate is used as therapy for arthritis, retinal vasculitis and chronic central nervous system lesions
  • Azathioprine (Azasan, Imuran),
  • Cyclosporine (Gengraf, Neoral, Sandimmune) and cyclophosphamide.
  • Tetracycline is used in the treatment of oral ulcers
  • Colchicine is used in the treatment of arthritis, oral ulcers, genital ulcers, erythema nodosum
  • Thalidomide is used to treat oral and genital ulcers
  • Dapsone is used as alternative therapy for oral and genital ulcers and for pseudofolliculitis
  • Pentoxifylline is used orally as therapy for oral ulcers, genital ulcers, pseudofolliculitis and erythema nodosum
  • Sulfasalazine is used as therapy for arthritis
  • Aspirin is used as therapy for arteritis, venous thrombosis and chronic progressive central nervous system lesions
  • MAGIC mouthwash is used to alleviate pain in the oral mucosa

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