Behçet's disease complicated by pylephlebitis and hepatic abscesses

A.C. Gelber1, L. Schachna1, L. Mitchell4, G. Schwartzman2, G. Hartnell2,3, J.F. Geschwind2,3

Departments of Medicine1, Radiology2, and Surgery3, Johns Hopkins University School of Medicine; and Department of Chemistry, University of Maryland4, Baltimore, Maryland, USA.

ABSTRACT
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. 

Key words
Behçet's disease, pylephlebitis, hepatic abscess, vasculitis.


Please address correspondence and reprint requests to: Allan C. Gelber, MD, 1830 East Monument St., Suite 7500, Baltimore, Maryland 21205, USA. 
E-mail: agelber@jhmi.edu

Clin Exp Rheumatol 2001; 19: S59-S61.
© Copyright Clinical and Experimental Rheumatology 2001.