Behcet Syndrome Workup

  • Author: C Egla Rabinovich, MD, MPH; Chief Editor: Lawrence K Jung, MD   more...
 
Updated: Apr 13, 2012
 

Laboratory Studies

  • No specific laboratory test result is diagnostic of Behçet syndrome.
    • Serum complement levels are within the reference range, except for just prior to eye or mucous membrane involvement, at which time they may be decreased.
    • Sedimentation rate or C-reactive protein may be elevated. Chronic anemia common, and a neutrophil leukocytosis is seen in about 15% of patients.
    • Human leukocyte antigen (HLA)-B51 may be present in patients of Asian, Mexican, or Middle Eastern descent.
    • Anticardiolipin antibodies are present in as many as 30% of patients.
  • Systemic lupus erythematosus and other vasculitic syndromes must be ruled out. Patients with Behcet syndrome have negative antinuclear and antineutrophilic cytoplasmic antibodies.
  • In patients with CNS findings, cerebral spinal fluid pleocytosis may be present.
  • In addition to thrombosis associated with antiphospholipid antibodies, thrombosis has been reported in Behçet syndrome associated with factor V Leiden mutations and with prothrombin G20210A mutations.
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Imaging Studies

  • Brain MRI and/or CT scanning for visualization of the neurological lesions is often helpful in patients with CNS involvement. Focal lesions may be observed anywhere in the CNS on the MRI, appearing as high signal on the T2-weighted images and low signal on the T1-weighted images. Flare images may be especially helpful. Enlargement of ventricles or subarachnoid spaces may be observed. However, the MRI findings of the brain may be normal even in the presence of neurologic involvement. Neuropsychologic testing results may be abnormal prior to any detectable lesions on neuro-imaging.
  • Angiography shows areas of aneurysm formation and thrombosis.
  • Echocardiography is useful in patients with murmurs because it is useful for diagnosing the valve vegetations and ventricular thrombi, which can occur in Behçet syndrome. MRI, T2-weighted images of brainstem involvement wMRI, T2-weighted images of brainstem involvement with meningoencephalitis in an 11-year-old girl with neurologic Behçet syndrome.
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Other Tests

  • Endoscopy of the GI tract is useful for detecting gastrointestinal ulcerations.
  • A thorough eye examination by an experienced ophthalmologist is essential. Consider fluorescein angiography for evaluation of retinal vessels. Follow-up visits with an ophthalmologist should be scheduled at least every 6-12 months.
  • Neuropsychologic testing may be useful with CNS involvement, revealing memory impairment or personality changes, and can be useful in monitoring neuropsychologic status.
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Histologic Findings

  • Behçet syndrome is diagnosed clinically, not by means of tissue evaluation. However, round cell infiltration may be found in cardiac valve lesions.
  • Biopsy of the buccal and genital ulcers reveals lymphocytic and plasma cell invasion in the prickle cell layer of the epidermis.
  • Dermal vessels are infiltrated with lymphocytes and plasma cells with immune deposits of immunoglobulin M (IgM) and C3. Occasionally, necrotizing vasculitis is observed. Histology of ulcers revealing neutrophilic infiltrHistology of ulcers revealing neutrophilic infiltrate and vasculitis.
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Contributor Information and Disclosures
Author

C Egla Rabinovich, MD, MPH  Associate Professor and Co-Division Chief, 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: Abbott Grant/research funds clincal trial; Pfizer Grant/research funds clinical trial; Centacor Grant/research funds clinical trial

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.

Specialty Editor Board

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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.

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.

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. Al-Dhibi H, Abouammoh M, Al-Harthi E, Al-Gaeed A, Larsson J, Abboud E, et al. Macular hole in Behçet's disease. Indian J Ophthalmol. Sep-Oct 2011;59(5):359-62. [Medline]. [Full Text].

  4. Al-Mujaini A, Wali UK. Frosted branch angiitis, neuroretinitis as initial ocular manifestation in Behçet disease. Indian J Ophthalmol. May-Jun 2011;59(3):240-1. [Medline]. [Full Text].

  5. Louali FE, Tamdy A, Soufiani A, Oukerraj L, Omari D, Bounjoum F, et al. Cardiac thrombosis as a manifestation of Behçet syndrome. Tex Heart Inst J. 2010;37(5):568-71. [Medline]. [Full Text].

  6. Seyahi E, Yurdakul S. Behçet's Syndrome and Thrombosis. Mediterr J Hematol Infect Dis. 2011;3(1):e2011026. [Medline]. [Full Text].

  7. 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].

  8. [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].

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

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

  11. 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].

  12. 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].

  13. 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].

  14. 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].

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

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

  17. [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].

  18. 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].

  19. 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].

  20. 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].

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

  22. 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].

  23. 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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MRI, T2-weighted images of brainstem involvement with meningoencephalitis in an 11-year-old girl with neurologic Behçet syndrome.
Histology of ulcers revealing neutrophilic infiltrate and vasculitis.
 
 
 
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