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Semin Arthritis Rheum. 2015 Apr;44(5):602-609. doi: 10.1016/j.semarthrit.2014.10.014. Epub 2014 Nov 1.

An outcome survey of 43 patients with Budd-Chiari syndrome due to Behçet's syndrome followed up at a single, dedicated center.

Author information

1
Division of Rheumatology, Department of Medicine, Cerrahpaşa Medical Faculty, University of Istanbul, Istanbul, Turkey 81310. Electronic address: eseyahi@yahoo.com.
2
Divison of Gastroenterology, Department of Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey.
3
Division of Rheumatology, Department of Medicine, Cerrahpaşa Medical Faculty, University of Istanbul, Istanbul, Turkey 81310.
4
Department of Radiology, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey.

Abstract

BACKGROUND:

Behçet's syndrome (BS) is a well-recognized cause of Budd-Chiari syndrome (BCS); however, information about its clinical characteristics and outcome is limited.

METHODS:

We reviewed the records of about 9000 patients with BS registered at the multidisciplinary Behçet's syndrome outpatient clinic at Cerrahpasa Medical Faculty between July 1977 and October 2013. We identified 43 (40 M/3 F) patients who were diagnosed as having BCS. Their outcome was evaluated between September 2012 and October 2013.

RESULTS:

In total, 33 patients (77%) had presented with liver-related symptoms (Group I), while 10 (23%) were asymptomatic for liver disease (Group II). This latter group had presented with symptoms related to the presence of major vessel disease such as fever, leg swelling, or dyspnea. The site of venous obstruction determined in 41 patients was inferior vena cava (IVC) and hepatic veins combined in 25 (61%), IVC alone in 12 (29%), and only hepatic veins in 4 patients (10%). The number of patients with concurrent obstruction in the hepatic veins and the IVC was less in Group II than in Group I (3/10 vs 22/31, p = 0.06). A total of 20 (19 M/1 F) patients (47%) had died at a median of 10 months after diagnosis. Mortality was significantly lower in Group II (10%) than in Group I (58%), (p = 0.011). By the end of the survey, 23 patients were alive, of whom 21 could be re-evaluated at the clinic.

CONCLUSIONS:

BCS associated with BS is usually due to IVC thrombosis with or without hepatic vein thrombosis. Silent cases exist and have a better prognosis. The mortality rate among the patients symptomatic for liver disease remains high.

KEYWORDS:

Behcet’s syndrome; Budd–Chiari syndrome; Hepatic Doppler USG; Hepatic veins; Inferior vena cava; Thrombosis

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