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Behçet's disease complicated by pylephlebitis and hepatic abscesses.
A C Gelber1, L Schachna, L Mitchell
1Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. agelber@jhmi.edu
Clinical and Experimental Rheumatology
|January 5, 2002
Summary
Behçet's disease can cause pylephlebitis, a rare septic thrombophlebitis of the portal vein. This case highlights a previously unrecognized complication involving intrahepatic abscesses and a diseased portal venous system.
Area of Science:
- Vascular Medicine
- Gastroenterology
- Rheumatology
Background:
- Behçet's disease is a rare multisystem inflammatory disorder.
- It is characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions.
- Vasculitis affecting various blood vessels is a key pathological feature.
Observation:
- A 22-year-old man with known Behçet's disease presented with fever, abdominal pain, weight loss, and diarrhea.
- Imaging revealed mesenteric arteritis and pylephlebitis (septic thrombophlebitis of the portal vein) with intrahepatic abscesses.
- Portal venography showed an extensively diseased, expanded, and obstructed portal venous system.
Findings:
- Blood cultures and portal vein aspirate identified polymicrobial flora.
- Percutaneous intraportal thrombolytic therapy and mechanical thrombectomy were performed to restore portal venous flow.
- Pylephlebitis is identified as a previously unrecognized complication of Behçet's disease.
Implications:
- This case suggests that pylephlebitis can arise from Behçet's disease-associated vasculitis.
- Ischemic injury and mucosal compromise of the bowel may predispose to this severe complication.
- Early recognition and intervention are crucial for managing this rare manifestation of Behçet's disease.