eMedicine Specialties > Pediatrics: General Medicine > Rheumatology

Behcet Syndrome: Follow-up

Author: C Egla Rabinovich, MD, MPH,, Assistant Professor and Co-Division Director, Department of Pediatrics, Division of Pediatric Rheumatology, Duke University Medical Center
Coauthor(s): Linda Wagner-Weiner, MD, Assistant Professor, Department of Pediatrics, University of Chicago
Contributor Information and Disclosures

Updated: Aug 24, 2009

Follow-up

Further Inpatient Care

  • Patients with Behçet syndrome may need to be admitted during acute flares for intravenous therapy or for diagnostic testing or surgical intervention.

Further Outpatient Care

  • Close follow-up care is warranted to monitor clinical status, including routine eye examinations.

Inpatient & Outpatient Medications

  • Medications (see Medication) vary depending on the clinical manifestations. Monitor drug side effects and efficacy closely.

Transfer

  • Transfer to a tertiary care center for diagnosis and intervention may be warranted, depending on the clinical symptomatology.

Deterrence/Prevention

  • No preventative measures are known for Behçet syndrome.

Complications

  • Complications include those caused by medications and the primary disease, and they vary depending on clinical presentation and disease manifestations.

Prognosis

  • Prognosis depends on clinical manifestations. The worst prognoses are associated with retinal vasculitis, leading to blindness; vascular aneurysm formation, with possible rupture; and neuro–Behçet syndrome, which may lead to dementia despite appropriate aggressive treatment.

Patient Education

  • Patients must be educated in disease manifestations, long-term prognosis, and medication side effects.

Miscellaneous

Medicolegal Pitfalls

  • Most of the drugs used to treat Behçet syndrome are immunosuppressive, with associated risk of serious life-threatening infections that patients should be warned about. In addition, cytotoxics increase the risk of malignancy, and communication of these risks should be clearly delineated and documented in the medical record.
 


More on Behcet Syndrome

Overview: Behcet Syndrome
Differential Diagnoses & Workup: Behcet Syndrome
Treatment & Medication: Behcet Syndrome
Follow-up: Behcet Syndrome
Multimedia: Behcet Syndrome
References
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References

  1. Al-Araji A, Kidd DP. Neuro-Behcet's disease: epidemiology, clinical characteristics, and management. Lancet Neurol. Feb 2009;8(2):192-204. [Medline].

  2. Ebert EC. Gastrointestinal manifestations of Behçet's disease. Dig Dis Sci. Feb 2009;54(2):201-7. [Medline].

  3. Ihle J, Kummerle-Deschner J, Orlikowsky T, Albert E, Niethammer D, Dannecker GE. Factor V Leiden and venous thrombosis in a 4-yr-old girl with Behcet's syndrome. Rheumatology (Oxford). Feb 2000;39(2):209-10. [Medline].

  4. [Guideline] Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. Dec 2008;67(12):1656-62. [Medline].

  5. Alpsoy E. Behcet's disease: treatment of mucocutaneous lesions. Clin Exp Rheumatol. Jul-Aug 2005;23(4):532-9. [Medline].

  6. Fresko I, Soy M, Hamuryudan V, et al. Genetic anticipation in Behcet's syndrome. Ann Rheum Dis. Jan 1998;57(1):45-8. [Medline].

  7. Gerber S, Biondi A, Dormont D, Wechsler B, Marsault C. Long-term MR follow-up of cerebral lesions in neuro-Behcet's disease. Neuroradiology. Nov 1996;38(8):761-8. [Medline].

  8. Kesen MR, Goldstein DA, Tessler HH. Uveitis associated with pediatric Behcet disease in the american midwest. Am J Ophthalmol. Dec 2008;146(6):819-27.e2. [Medline].

  9. Kikuchi H, Aramaki K, Hirohata S. Effect of infliximab in progressive neuro-Behcet's syndrome. J Neurol Sci. Sep 15 2008;272(1-2):99-105. [Medline].

  10. Kone-Paut I, Geisler I, Wechsler B, et al. Familial aggregation in Behcet's disease: high frequency in siblings and parents of pediatric probands. J Pediatr. Jul 1999;135(1):89-93. [Medline].

  11. Marshall SE. Behcet's disease. Best Pract Res Clin Rheumatol. Jun 2004;18(3):291-311. [Medline].

  12. Martini A. Behcet's disease and Takayasu's disease in children. Curr Opin Rheumatol. Sep 1995;7(5):449-54. [Medline].

  13. [Best Evidence] Melikoglu M, Fresko I, Mat C, et al. Short-term trial of etanercept in Behçet's disease: a double blind, placebo controlled study. J Rheumatol. Jan 2005;32(1):98-105. [Medline].

  14. Nakamura S, Ohno S. Anti-tumor necrosis factor alpha antibody in the treatment of Behcet's disease. Int Ophthalmol Clin. 2005;45(2):179-89. [Medline].

  15. Oktem-Tanor O, Baykan-Kurt B, Gurvit IH, et al. Neuropsychological follow-up of 12 patients with neuro-Behcet disease. J Neurol. Feb 1999;246(2):113-9. [Medline].

  16. Rakavor Y, Adar H, Tal I, et al. Behcet Disease: Long-term follow-up of three children and review of the literature. Pediatrics. 1989;83:986-92. [Medline].

  17. Sakane T, Takeno M, Suzuki N, Inaba G. Behcet's disease. N Engl J Med. Oct 21 1999;341(17):1284-91. [Medline].

  18. Tabbara KF, Al-Hemidan AI. Infliximab effects compared to conventional therapy in the management of retinal vasculitis in Behçet disease. Am J Ophthalmol. Dec 2008;146(6):845-50.e1. [Medline].

  19. Yazici H, Pazarli H, Barnes CG, et al. A controlled trial of azathioprine in Behcet's syndrome. N Engl J Med. Feb 1 1990;322(5):281-5. [Medline].

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Further Reading

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Keywords

Behçet syndrome, Behçet's disease, Behçet disease, Behcet syndrome, Behcet's syndrome, Adamantiades-Behçet's disease, Adamantiades-Behçet disease, blindness, aphthous ulcers, genital ulcers, vasculitis, Budd-Chiari syndrome, eye lesions, skin lesions, erythema nodosum, pseudofolliculitis, uveitis, cutaneous pustular vasculitis, synovitis, meningoencephalitis, treatment, diagnosis 

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Contributor Information and Disclosures

Author

C Egla Rabinovich, MD, MPH,, Assistant Professor and Co-Division Director, Department of Pediatrics, Division of Pediatric Rheumatology, Duke University Medical Center
C Egla Rabinovich, MD, MPH, is a member of the following medical societies: American College of Rheumatology
Disclosure: Nothing to disclose.

Coauthor(s)

Linda Wagner-Weiner, MD, Assistant Professor, Department of Pediatrics, University of Chicago
Linda Wagner-Weiner, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Rheumatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Thomas JA Lehman, MD, FAAP, FACR, Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Rheumatology, Weill-Cornell University; Chief, Hospital for Special Surgery
Thomas JA Lehman, MD, FAAP, FACR is a member of the following medical societies: PM American Allergy Society
Disclosure: Nothing to disclose.

CME Editor

Paul D Petry, DO, FACOP, FAAP, Consulting Staff, Freeman Pediatric Care, Freeman Health System
Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association
Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD, Chief, Division of Pediatric Rheumatology, Children's National Medical Center
Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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