Behçet Disease: Multisystem Imaging Review
A practical radiology review of Behçet Disease: Multisystem Imaging Review, focused on imaging findings, differential diagnosis, reporting points, and high-yield teaching pearls.
A practical radiology review of Behçet Disease: Multisystem Imaging Review, focused on imaging findings, differential diagnosis, reporting points, and high-yield teaching pearls.
- Etiology & Diagnosis
- Chronic relapsing vasculitis of all vessel sizes
- Dx = recurrent oral ulcers + ≥2 (genital ulcers / uveitis / skin lesions / pathergy +)
- Imaging used for systemic involvement & complications, not primary diagnosis
Neurologic (Neuro-Behçet)
- Two phenotypes: parenchymal vs non-parenchymal (CVST / IH)
Parenchymal (brainstem-dominant)
- ▶ Brainstem > thalamus > white matter
- ▶ T2/FLAIR high signal; +/- enhancement if acute
- ▶ Chronic: atrophy, T1 black holes
- Mimics: MS / ADEM / tumor / abscess
Non-parenchymal
- ▶ CVST → empty delta / loss of flow void / venous infarct
- ▶ Intracranial hypertension → papilledema ± normal MRI
- Vascular Involvement (Vasculo-Behçet)
- Venous >>> Arterial
- Venous: DVT, IVC/SVC thrombosis, Budd–Chiari, portal cavernoma
- Arterial: saccular aneurysms (aorta, pulmonary, femoral) ± rupture risk
- Aneurysm recurrence after surgery is common
Phenotypes
- Parenchymal NBD — inflammatory meningo-encephalitis (brainstem-dominant)
- Non-parenchymal NBD — venous disease (CVST / IH) without parenchymal inflammation
- Mixed — CVST + parenchymal lesions
Parenchymal NBD — Where it lives
- Brainstem → midbrain & pons = MOST classic
- Deep gray → thalamus / basal ganglia
- White matter → periventricular & deep; spinal cord also possible
- Less typical: cortical, tumor-like, solitary “mass-like” lesions
Non-Parenchymal NBD
- Cerebral venous sinus thrombosis (CVST) → most typical
- CT: “empty-delta”
- MRV/SWI: loss of flow void / absent sinus signal
- Intracranial hypertension ± papilledema
- MRI may be normal or show secondary venous changes
Neuro-clinical Correlation
- Parenchymal → pyramidal signs, cranial nerve palsy, ocular involvement, dysarthria
- Non-parenchymal → headache, papilledema, CVST, high ICP
- Mixed → headache + pyramidal ± fever/diplopia
Thoracic
- Pulmonary artery aneurysm or thrombosis
- SVC syndrome → downhill varices
- PE / infarction / pericardial vasculitis
GI Tract
- Deep “punched-out” ileocecal ulcers ± fistula/perforation
- Less often: stomach/esophagus ulcers; post-op recurrence frequent
Cardiac
- Intracardiac thrombus, endomyocardial fibrosis, sinus of Valsalva aneurysm
- Rare but life-threatening
Treatment Pearls
- Steroids + immunosuppressives (esp. neurologic/vascular)
- Avoid early surgery when possible — high recurrence & pseudoaneurysm risk
Clinical use note
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