Behçet's disease in Budd-Chiari Syndrome

Desbois, Anne Claire; Rautou, Pierre‐Emmanuel; Plessier, Aurélie; Belmatoug, Nadia; Chambrun, Marc Pineton de; Wechsler, B.; Resche‐Rigon, M.; Fantin, Bruno; Valla, Dominique; Cacoub, P.; Saadoun, David

doi:10.1016/j.lpm.2013.02.061

Cited by 5 publications

(10 citation statements)

References 8 publications

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“…The inferior vena cava was involved in 80% of the cases; this is the main characteristic of BCS in BD, which has also been reported in other studies, [5–11] and is uncommon in BCS due to other causes. [13,14]…”

Section: Discussionsupporting

confidence: 77%

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Budd–Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco

et al. 2022

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Budd-Chiari syndrome (BCS) is considered a rare but serious complication of Behçet's disease (BD). This study was performed to define the prevalence, clinical and biological features, treatment, and clinical course of BSC associated with BD in a Moroccan population. We retrospectively analyzed the medical records of 1578 patients fulfilling the international diagnostic criteria for BD, including those with BSC. Eighteen male and 3 female patients, with a mean age of 36 ± 8.6 years. The inferior vena cava was involved in 81% (n = 17) of cases. All forms of BCS were noted: the chronic form in 52.4% (n = 11), the subacute form in 38% (n = 8), and the fulminant form (2 cases). Ascites was the main clinical sign and was present in 62% of patients (n = 13). Other venous thromboses (superior vena cava and lower limbs) were associated with BSC in 52.4% of patients (n = 11). Arterial involvement was noted in 28.6% (n = 6). Cardiac manifestations were present in 19% (n = 4) of the patients. All the patients received anticoagulants associated with corticosteroids. Immunosuppressants were used in 95% (n = 20). One patient received infliximab. Severe complications were noted in 38% (n = 8) of patients (digestive bleeding, confusion, infections and liver failure). Four patients have died during the study period. BCS in patients with BD is not uncommon and can be life threatening. It is frequently associated with other vascular manifestations that can be difficult to treat, particularly in the presence of pulmonary artery aneurysms. Prognosis improved with the use of immunosuppressants. Biologics can be promising in the early stages.

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Section: Discussionsupporting

confidence: 77%

“…We also believe that we should always search for BD in patients with BCS, especially in young men. It is well established that vascular involvement is more common in young males, [5][6][7][8][9][10] as the majority of our patients.…”

Section: Discussionmentioning

confidence: 84%

Budd–Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco

et al. 2022

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“…Lower extremity oedema, abdominal collaterals and abdominal extension were presented more frequently in BS patients with IVCS. In a literature review, IVC thrombosis was documented in 91% of BS patients with BCS (27). Unlike BCS with other causes, hepatic venous outflow obstruction in BS is related to the thrombosis of the hepatic and suprahepatic segments of IVC.…”

Section: Discussionmentioning

confidence: 99%

The clinical features, image findings and risk factors of vena cava syndrome in Behçet's syndrome

Zhou

Wang

Hou

et al. 2022

Clinical and Experimental Rheumatology

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Objective To investigate the clinical features, image findings, and potential risk factors of vena cava syndrome (VCS) inBehçet's syndrome (BS). MethodsWe conducted a case-control study in our BS registry database from 2012 to 2021. Fifty-five BS patients with VCS were enrolled in the case group, and two BS patients without VCS were selected as controls for each VCS case using risk-set-sampling. Multivariable logistic regression was used to detect the risk factors of VCS, and the outcome of these patients was also analysed. We also conducted an exploratory study to evaluate spectral computed tomography (CT) imaging in detecting thrombosis in BS patients with inferior VCS (IVCS).

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“…BD is a well-recognized cause of Budd-Chiari syndrome. 1 Aseptic abscesses are a diagnosis of exclusion, reached by the criteria suggested by André et al, 2 and have been reported in association with inflammatory bowel disease, Sweet's syndrome, and pyoderma gangrenosum.…”

Section: Answer To: Image 4 (Page 495): Multiple Aseptic Abscesses Ofmentioning

confidence: 99%

Persistent Fever and Abdominal Pain in a Young Woman With Budd-Chiari Syndrome

2018

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A 23-year-old woman with Budd-Chiari syndrome in treatment with warfarin was admitted to our unit because of the persistence of fever, severe abdominal pain, diarrhea, and marked weight loss. Her medical history included recurrent oral and genital aphthous ulcers, arthritis, and erythema nodosum. Two months before admission, she underwent urgent left hemicolectomy for a massive intestinal hemorrhage. Treatment with corticosteroids was started after diagnosing probable Behçet's disease (BD). At admission, the patient presented with tachycardia and hypotension; her abdomen was mildly distended, with a generalized tenderness to palpation without rebound or guarding. The methylprednisolone dose had recently been tapered to 4 mg/d because of hirsutism. Laboratory tests revealed neutrophilic leukocytosis, mild thrombocytosis, severe microcytic anemia, mild elevation of the International Normalized Ratio, alkaline phosphatase, and gamma-glutamyl transferase, and a marked increase of C-reactive protein and the erythrocyte sedimentation rate. A contrast-enhanced abdominal computed tomography scan demonstrated spread areas of low-attenuation with "rim enhancement" in the liver and spleen, compatible with multiple abscesses (Figure A, B), necrotic celiac and paraaortic lymph nodes (Figure C), almost complete thrombosis of the right and left hepatic veins, and thrombosis of the distal tract of the splenic vein. Steroid therapy was discontinued immediately. Despite the negativity of blood, urine, and stool cultures, an empirical antibiotic therapy with piperacillin/tazobactam and teicoplanin was started. Nevertheless, there was a sudden worsening of the hemodynamic state, unresponsive to a fill fluid challenge, and therefore, it was necessary to transfer the patient to the intensive care unit.

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Behçet's disease in Budd-Chiari Syndrome

Cited by 5 publications

References 8 publications

Budd–Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco

Budd–Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco

The clinical features, image findings and risk factors of vena cava syndrome in Behçet's syndrome

Persistent Fever and Abdominal Pain in a Young Woman With Budd-Chiari Syndrome

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