Session Title: Vasculitis
Session Type: Abstract Submissions (ACR)
Behçet’s syndrome (BS) is a well recognized cause of Budd–Chiari syndrome (BCS), however, information about its clinical characteristics and outcome is limited. We had presented the outcome of 40 patients with BCS due to BS at the ACR 2013 meeting (1). In further analyses, we better understood that there were patients in whom BCS could exist without the presence of any liver related symptoms/signs. In the current study we compared demographic, clinical, radiologic and prognostic characteristics of these patients to those presented with overt liver related symptoms.
We reviewed the records of about 9000 patients with BS who were registered at the multidisciplinary Behçet’s syndrome outpatient clinic at Cerrahpasa Medical Faculty between July 1977 and October 2013. We identified 43 (40 male/ 3 female) patients who were diagnosed as having BCS. Their outcome was evaluated between September 2012 and October 2013.
43 (40 male/ 3 female) patients with BCS were identified. Thirty-three patients (77 %) had presented with liver related symptoms such as ascites and abdominal swelling for a median duration of 1 month [IQR:0.5-2.5 months]. These patients were defined as Group I. On the other hand, 10 (21%) were silent for liver disease at presentation and were found to have BCS while being investigated for fever (n=4), bilateral diffuse leg swelling (n = 4), and pulmonary symptoms like hemoptysis or dyspnea (n =4). None had liver dysfunction related symptoms/signs. These patients were classified as Group II. All were diagnosed after 1990. Group II patients were somewhat older (mean age: 32.2 vs 28.7, p=0.26) and had longer disease duration of BS at the onset of BCS (median: 5.1 vs 2.7, p =0.18) than Group I patients. The level of hepatic venous outflow obstruction in Group II involved less frequently the combined IVC and hepatic veins (30 %) while was more likely to involve isolated hepatic veins (30 %) or isolated IVC (40 %) when compared to Group I (76 %, 26 % and 3 %, respectively) (p =0.05). Besides that, the anatomical distribution of vessels other than those involved in BCS, and the clinical characteristics of BS were not very different than Group I. By the end of the survey, there was only 1 death in Group II, which was not due to hepatic failure. Mortality was significantly lower among Group I patients (1/10) as compared to Group II (19/33, 58 %), (p =0.025).By the end of the survey, only 1 (1/13) in Group I, had ascites, while in the remaining surviving patients in Group I ascites had resolved within 6 to 8 months after the first visit. A final hepatic Doppler USG indicated that, the frequency of recanalisation and collateral formation was similar between the patients in Group I and II.
There is subgoup of patients in whom, BCS may exist without the presence of liver related symptoms/signs and these patients have a good prognosis . These patients could pass unnoticed, unless one looks carefully for hepatic veins or intra or suprahepatic segments of the IVC. The fact that, 8 of these10 patients were diagnosed after 2000, suggests that this subgroup was recognized after the recent availability of imaging techniques.
Seyahi E et al. ACR 2013
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