Behcet Disease Treatment & Management

  • Author: Augusto C Posadas, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Aug 26, 2011
 

Medical Care

Oral ulcerations can be successfully treated with topical steroids or sucralfate solution. Colchicine has also been used to prevent mucocutaneous relapse.[22, 23]

For severe mucocutaneous lesions, systemic corticosteroids, azathioprine, pentoxifylline, dapsone, interferon-alpha, colchicine, and thalidomide have demonstrated benefit.

For genital ulceration, topical and intralesional steroids can be used. Topical sucralfate also has demonstrated benefit.

GI lesions are primarily treated with 5-ASA derivatives, including sulfasalazine or mesalamine. However, upon lack of response, systemic corticosteroids can be used with eventual taper as dictated by decreasing C-reactive protein values on surveillance. Upon development of steroid dependence, azathioprine can be used adjunctively in an attempt to decrease the need for glucocorticoids.

Pulmonary arterial aneurysms are treated with cyclophosphamide.

Joint involvement may respond to prednisone, local corticosteroid injections, and nonsteroidal anti-inflammatory drugs (NSAIDs). Colchicine, sulfasalazine, and interferon-alpha are also used. Levamisole and azathioprine serve as alternative modes of therapy.

Multiple modes of therapy have been studied for ocular symptoms, and a good response has been demonstrated with interferon therapy. TNF-inhibitor therapy with infliximab (Remicade) and etanercept (Enbrel) has also demonstrated favorable response in treating rapidly progressive anterior and posterior uveitis. Cyclosporine has been well supported in the literature as an effective therapeutic measure.

Erythema nodosum is a special circumstance and may be treated with colchicine or dapsone.

CNS disease is usually treated with systemic corticosteroids, chlorambucil, or cyclophosphamide.

Thrombotic events are treated with systemic anticoagulation.

TNF-inhibitor therapy with infliximab and etanercept has also demonstrated varying degrees of success in treating cases of severe GI, ocular, and CNS manifestations of Behçet disease and has been shown to have the added benefit of improving mucocutaneous manifestations and polyarthritis.[24, 25]

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Surgical Care

GI presentations that require surgical intervention include intestinal stenosis, lesions unresponsive to medical therapy, fistula formation, perforation, and severe bleeding.

Pulmonary aneurysms and areas that incur ischemic damage due to vasculitis or thrombosis may require resection.

Ventricular aneurysms, coronary thrombosis, and endocardial fibrosis are occasionally amenable to surgery.

Glaucoma, cataracts, and retinal detachment occasionally warrant surgical intervention.

Neurosurgery may be required to correct some CNS aneurysms and clots.

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Consultations

  • Rheumatologist
  • Urologist for genital and urologic lesions
  • Neurologist for CNS involvement
  • Ophthalmologist for ocular disease
  • Gastroenterologist for intestinal disease
  • Dermatologist for possible help with recurrent skin lesions
  • Surgeon, when indicated
  • Nephrologist for proteinuria or hematuria
  • Pulmonologist or cardiologist in rare cases of intracardiac or pulmonary thrombosis and aneurysms
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Diet

  • No general dietary recommendations exist.
  • Patients with severe bowel involvement are advised to follow GI recommendations given to patients with inflammatory bowel disease, often requiring total parenteral nutrition.
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Activity

Activity is suggested as tolerated and may be limited owing to systemic symptoms or arthritis.

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Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Augusto C Posadas, MD  Rheumatologist, Saguaro Physician Group, Tucson, AZ

Disclosure: Abbott Honoraria Speaking and teaching

Coauthor(s)

Jeffrey R Lisse, MD, FACP  Professor, Department of Internal Medicine, Chief, Section of Rheumatology, University of Arizona School of Medicine

Jeffrey R Lisse, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American College of Rheumatology, American Geriatrics Society, and Sigma Xi

Disclosure: Genentech Consulting fee Consulting; Centacor Consulting fee Consulting; Novartis Consulting fee Review panel membership

Specialty Editor Board

Kristine M Lohr, MD, MS  Professor, Department of Internal Medicine, Center for the Advancement of Women's Health and Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Lawrence H Brent, MD  Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching; Takeda Honoraria Speaking and teaching; UCB Speaking and teaching; Omnicare Consulting fee Consulting; Centocor Consulting fee Consulting

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

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Oral aphthous ulcers secondary to Behçet disease.
 
 
 
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