Behçet's disease complicated by pylephlebitis and hepatic abscesses.
A 22 year old man presented with fever, abdominal pain, weight loss and diarrhea. Past medical history revealed recurrent aseptic meningitis, uveitis, and erythema nodosum. Further inquiry unveiled a prominent history of oral aphthous ulcers; all features of Behçet's disease. Imaging revealed mesenteric arteritis and pylephlebitis, septic thrombophlebitis of the portal vein, a previously unrecognized complication of Behçet's disease, with multiple intrahepatic abscesses. Portal venography demonstrated an extensively diseased, expanded, and obstructed portal venous system. Blood cultures and portal vein aspirate yielded polymicrobial flora. Percutaneous intraportal thrombolytic therapy and mechanical thrombectomy were attempted to restore flow to the portal venous system. This distinctly rare manifestation of Behçet's Disease, pylephlebitis, may result from ischemic injury and structural compromise of the bowel mucosa, resulting from underlying vasculitis.
Gelber
A C
AC
Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. agelber@jhmi.edu
Schachna
L
L
Mitchell
L
L
Schwartzman
G
G
Hartnell
G
G
Geschwind
J F
JF
D000328:Adult; D001528:Behcet Syndrome / Q000150:complications*; D004927:Escherichia coli Infections / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D008100:Liver Abscess / Q000000981:diagnostic imaging / Q000209:etiology*; D008297:Male; D017538:Mesenteric Artery, Superior / Q000000981:diagnostic imaging; D011169:Portal Vein / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed; D020246:Venous Thrombosis / Q000000981:diagnostic imaging / Q000209:etiology*