Ophthalmologic Manifestations of Behcet Disease Workup

  • Author: Mounir Bashour, MD, CM, FRCS(C), PhD, FACS; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Apr 4, 2012
 

Laboratory Studies

  • The diagnosis of Behçet disease is based on clinical findings rather than on specific laboratory test results. Some tests are useful adjuncts in the evaluation of patients who have Behçet disease.
  • The pathergy test (or Behçetine test) may be useful in patient evaluation; the test result is a nonspecific inflammatory reaction to a needle prick or an intradermal injection of saline. The reaction, which varies from an indurated erythema to pustule formation, occurs in 40-60% of patients who have Behçet disease.
  • Several other laboratory tests may help in the evaluation of a patient who has Behçet disease. During episodes of acute inflammation, patients may have high erythrocyte sedimentation rates, high C-reactive protein levels, or an increased number of peripheral leukocytes.
  • In neuro-Behçet disease, an analysis of cerebrospinal fluid reveals pleocytosis and elevated protein levels.[10]
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Imaging Studies

  • Fundus fluorescein angiography shows diffuse retinal vascular leakage and occlusion of retinal vessels.
    • Fluorescein leakage from retinal vessels may be seen before any clinical signs of vasculitis.
    • During acute inflammation, retinal vascular leakage is prominent, especially in the radial peripapillary area.
    • Affected retinal and optic nerve vessels leak fluorescein profusely during early transit and their walls stain in late transit.
    • Fluorescein angiography also may reveal macular ischemia and cystoid macular edema.
  • In neuro-Behçet disease, magnetic resonance imaging (MRI) is the imaging study of choice and often reveals iso-hypointense lesions in T1-weighted images and hyperintense lesions in T2-weighted images, mostly in the mesodiencephalic junction, cerebellar peduncles, and other parts of the brainstem.[10]
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Histologic Findings

Histopathologic changes include necrotizing, leukocytoclastic, and obliterative vasculitis, which affect arteries and veins of all sizes and are probably immune-complex mediated. Only a few eyes that have had active disease have been examined histologically. Vasculitis is the key feature of Behçet disease. Underlying changes seen in the eye are similar to those that occur in other organs of the body.

During acute inflammation, the iris, the ciliary body, and the choroid show diffuse infiltration with neutrophils. In late stages, a proliferation of collagen fibers, thickening of the choroid, formation of cyclitic membrane, and sometimes hypotonia and phthisis bulbi are noted. Lymphocytic and plasma cell infiltration occurs during remission. Of all ocular tissues, the retina suffers the most damage. In the phase of acute inflammation, severe vasculitis with marked infiltration of leukocytes in and around blood vessels is seen. Recent and old hemorrhages are present. Retinal vessels have thickened basement membranes with swollen endothelial cells, which can lead to thrombus formation and vascular obliteration.

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Contributor Information and Disclosures
Author

Mounir Bashour, MD, CM, FRCS(C), PhD, FACS  Assistant Professor of Ophthalmology, McGill University; Clinical Assistant Professor of Ophthalmology, Sherbrooke University; Medical Director, Cornea Laser and Lasik MD

Mounir Bashour, MD, CM, FRCS(C), PhD, FACS is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American College of International Physicians, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, American Society of Mechanical Engineers, American Society of Ophthalmic Plastic and Reconstructive Surgery, Biomedical Engineering Society, Canadian Medical Association, Canadian Ophthalmological Society, Contact Lens Association of Ophthalmologists, International College of Surgeons US Section, Ontario Medical Association, Quebec Medical Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

John D Sheppard Jr, MD, MMSc  Professor of Ophthalmology, Microbiology and Molecular Biology, Clinical Director, Thomas R Lee Center for Ocular Pharmacology, Ophthalmology Residency Research Program Director, Eastern Virginia Medical School; President, Virginia Eye Consultants

John D Sheppard Jr, MD, MMSc is a member of the following medical societies: American Academy of Ophthalmology, American Society for Microbiology, American Society of Cataract and Refractive Surgery, American Uveitis Society, and Association for Research in Vision and Ophthalmology

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

R Christopher Walton, MD  Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, Assistant Dean for Graduate Medical Education, University of Tennessee College of Medicine; Consulting Staff, Regional Medical Center, Memphis Veterans Affairs Medical Center, St Jude Children's Research Hospital

R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Healthcare Executives, American Uveitis Society, Association for Research in Vision and Ophthalmology, and Retina Society

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

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Treatment modalities currently used in Behçet disease according to clinical symptoms.
 
 
 
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