eMedicine Specialties > Rheumatology > Vasculitis

Behcet Disease: Differential Diagnoses & Workup

Author: Augusto C Posadas, MD, Fellow, Department of Medicine, Section of Rheumatology, University of Arizona School of Medicine
Coauthor(s): Jeffrey R Lisse, MD, FACP, Professor, Department of Internal Medicine, Chief, Section of Rheumatology, University of Arizona School of Medicine
Contributor Information and Disclosures

Updated: Jun 22, 2009

Differential Diagnoses

Amyloidosis, AA (Inflammatory)
Systemic Lupus Erythematosus
Antiphospholipid Syndrome
Wegener Granulomatosis
Inflammatory Bowel Disease
Paraneoplastic Syndromes
Polyarteritis Nodosa

Other Problems to Be Considered

Malignancy
HLA-B27–associated syndromes (ankylosing spondylitis, reactive arthritis, psoriatic arthritis)
HIV/AIDS
Hypercoagulable states (eg, protein C and S deficiency, factor V Leiden, hyperhomocysteinemia, prothrombin deficiency)
Alternate causes of uveitis
Viral and bacterial infections (eg, HSV infection, chancroid)

Workup

Laboratory Studies

  • Laboratory findings
    • Laboratory findings are nonspecific and reflect the inflammatory state.
    • C-reactive protein levels, erythrocyte sedimentation rate (ESR), leukocyte count, complement components, and acute-phase reactants may all be elevated during an acute attack.
    • Levels of IgA, IgG, alpha-2 globulin, IgM, and immune complexes are occasionally elevated.
    • None of these findings is specific for the diagnosis of Behçet disease, but such findings can corroborate active disease.
  • Antiphospholipid antibodies: These include lupus anticoagulant, dilute Russell viper venom test (DRVVT), and anticardiolipin antibodies. Although uncommon in Behçet disease, they are worth pursuing to rule out alternate causes of thrombosis.
  • Up to one third of patients with Behçet disease who have thrombosis are found to have factor V Leiden–deficiency mutations. Therefore, this, APL antibody, and other causes of hypercoagulability should be ruled out as contributing factors to thrombosis formation.
  • Antineutrophil cytoplasmic antibody: Occasionally, patients are found with positive test results for perinuclear antineutrophil cytoplasmic (p-ANCA) antibody, although positive or negative results on this test do not change prognosis or therapy.
  • Synovial fluid: Synovial fluid usually is cloudy with variable viscosity, and the WBC counts are 300-36,000/µL (either noninflammatory or inflammatory). Polymorphonuclear leukocytes and protein elevations are the predominant findings, and glucose levels are near normal. Thus, because synovial fluid merely demonstrates general inflammation, examination serves only to rule out the presence of aseptic joint, crystal-induced arthropathy, or other alternate identifiable cause in patients with Behçet disease.
  • Cerebrospinal fluid: These findings may show local inflammation with increased WBC counts, lymphocyte predominance, and elevated protein levels, as well as Ig levels and Ig index that reflect local production of Ig. Opening pressures are very high in some patients.

Imaging Studies

  • Radiography, MRI, and CT scanning: Sacroiliitis may be observed on a radiograph, MRI, or CT scan.
  • Brain CT scanning: Acute areas of ischemia can be identified.
  • Brain MRI/magnetic resonance angiography (MRI/MRA): Cerebral vasculopathy and acute/subacute areas of ischemia can be identified.
  • Single-photon emission computed tomography (SPECT): This has been used to identify areas of cerebral hypoperfusion in Italian, Spanish, and Turkish studies among pediatric, adolescent, and adult populations.
  • Angiography: This test may be used to evaluate for aneurysms.

Other Tests

  • Pathergy test: Minor skin trauma induces an inflammatory papule or pustule within 24-48 hours.

Procedures

  • As with laboratory studies, samples obtained via arthrocentesis and lumbar puncture are used primarily to rule out alternate causes of disease presentation, as they normally demonstrate nonspecific inflammatory findings.
  • Similarly, skin biopsy results often reflect nondiagnostic findings but can be used to differentiate alternate disease entities.

Histologic Findings

Although no specific histologic findings characterize Behçet disease, biopsy samples of affected tissue often reveal leukocytoclastic vasculitis and perivascular infiltration. CNS lesions may demonstrate meningeal and cerebral inflammation, cerebral atrophy, and encephalomalacia. Thrombosis commonly develops in affected areas and must be distinguished from vasculitis as a precipitating cause for organ-specific symptoms. Other organ-system findings include the following:

  • Skin - Erythema nodosum lesions with characteristic findings and occasional granulomas; folliculitis; leukocytoclastic vasculitis; dermal inflammation and perivascular infiltrates; fibrosis; and mucosal lesions, including aggregated intravascular conglomerates of neutrophils, endothelial cell swelling, fibrinoid necrosis, and a mixed perivascular infiltrate (Pathergy test reveals mononuclear cell infiltrates and keratinocytes.)
  • Eye - Cataracts, posterior and anterior uveitis, retinal vasculitis and thrombosis, cytoid macular degeneration, retinal detachment, and lymphocytic infiltrates in the iris (even during clinical remissions)
  • Brain - Infarctions due to vasculitis or thrombosis, meningoencephalitis, lymphocytic meningeal infiltration, or demyelinization
  • Joint - Superficial inflammatory synovial infiltrates, mainly polymorphonuclear lymphocytes, and deposition of IgG in the synovium
  • GI tract - Ulcerations from the buccal mucosa to the anus, intestinal perforation, peritonitis, infiltration with polymorphonuclear leukocytes and lymphocytes, hepatitis, cholecystitis, and pancreatitis
  • Heart - Pericarditis, myocarditis, endocarditis, coronary arteritis, and myocardial fibrosis
  • Lung - Serositis and vasculitis
  • Kidneys - Glomerulonephritis

More on Behcet Disease

Overview: Behcet Disease
Differential Diagnoses & Workup: Behcet Disease
Treatment & Medication: Behcet Disease
Follow-up: Behcet Disease
Multimedia: Behcet Disease
References
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Further Reading

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Keywords

Behçet disease, Behcet disease, Behçet syndrome, Behcet syndrome, Behçet's disease, Adamantiades-Behçet disease, BD, inflammatory arthritis, sacroiliitis, ulcerative lesions, aphthous ulcers, oral ulcers, oral lesions, skin lesions, genital ulcers, phlebitis, uveitis, iritis, hypopyon, retinal vasculitis, erythema nodosum, pseudofolliculitis, papulopustular lesions, pathergy, vasculopathy, superficial thrombophlebitis, deep venous thrombophlebitis, Budd-Chiari syndrome, mouth and genital ulcers with inflamed cartilage, MAGIC, autoimmune disease

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Contributor Information and Disclosures

Author

Augusto C Posadas, MD, Fellow, Department of Medicine, Section of Rheumatology, University of Arizona School of Medicine
Disclosure: Nothing to disclose.

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: Nothing to disclose.

Medical Editor

Kristine M Lohr, MD, MS, Program Director, Professor, Department of Internal Medicine, Division of Rheumatology and Women's Health, University of Kentucky School of Medicine
Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology, and American Medical Women's Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Lawrence H Brent, MD, Associate Professor of Medicine, 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 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; Wyeth Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching

CME Editor

Alex J Mechaber, MD, FACP, 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, Professor of Medicine, Temple University 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: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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