Laboratory Studies
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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.
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Systemic lupus erythematosus and other vasculitic syndromes must be ruled out. Patients with Behcet syndrome have negative antinuclear and antineutrophilic cytoplasmic antibodies.
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In patients with CNS findings, cerebral spinal fluid pleocytosis may be present.
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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.
Imaging Studies
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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.
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Angiography shows areas of aneurysm formation and thrombosis.
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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 with meningoencephalitis in an 11-year-old girl with neurologic Behçet syndrome.
Other Tests
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Endoscopy of the GI tract is useful for detecting gastrointestinal ulcerations.
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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.
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Neuropsychologic testing may be useful with CNS involvement, revealing memory impairment or personality changes, and can be useful in monitoring neuropsychologic status.
Histologic Findings
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Behçet syndrome is diagnosed clinically, not by means of tissue evaluation. However, round cell infiltration may be found in cardiac valve lesions.
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Biopsy of the buccal and genital ulcers reveals lymphocytic and plasma cell invasion in the prickle cell layer of the epidermis.
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Dermal vessels are infiltrated with lymphocytes and plasma cells with immune deposits of immunoglobulin M (IgM) and C3. Occasionally, necrotizing vasculitis is observed.
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MRI, T2-weighted images of brainstem involvement with meningoencephalitis in an 11-year-old girl with neurologic Behçet syndrome.
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Histology of ulcers revealing neutrophilic infiltrate and vasculitis.
