Budd–Chiari syndrome (BCS) is characterized by an obstruction of hepatic venous outflow from small hepatic veins to inferior vena cava, caused by acute thrombosis or its fibrous sequellae. An ...underlying myeloproliferative neoplasm is present in 50% of cases. Clinical manifestations are widely variable, from asymptomatic to fulminant episodes. Long-term complications range from cirrhosis to hepatocellular carcinoma. Behçet’s disease (BD) is a rare recurrent inflammatory multisystemic disorder characterized by recurrent skin-mucosa lesions and systemic involvement. Vascular involvement is observed in up to 40% of the patients with BD, and it is one of the major causes of mortality and morbidity. BCS is a rare complication of BD with a frequency of < 5% among patients with vascular involvement. Immunosuppressive treatment is the cornerstone for the management of vascular involvement in BD, while anticoagulant therapy has been an issue of debate. Transjugular intrahepatic portosystemic shunt (TIPS) in severe cases of BCS—of all causes— improves survival. However, there is scarce evidence about the role of TIPS in the setting of BCS in BD. We present a case of a vascular Behçet’s disease associated with chronic Budd–Chiari syndrome with progression of thrombosis despite adequate anticoagulant and immunosuppressive treatment, successfully managed with TIPS.
Abstract
To analyze the frequency and clinical phenotype of neurosarcoidosis (NS) in one of the largest nationwide cohorts of patients with sarcoidosis reported from southern Europe. NS was evaluated ...according to the Diagnostic Criteria for Central Nervous System and Peripheral Nervous System Sarcoidosis recently proposed by Stern et al. Pathologic confirmation of granulomatous disease was used to subclassify NS into definite (confirmation in neurological tissue), probable (confirmation in extraneurological tissue) and possible (no histopathological confirmation of the disease). Of the 1532 patients included in the cohort, 85 (5.5%) fulfilled the Stern criteria for NS (49 women, mean age at diagnosis of NS of 47.6 years, 91% White). These patients developed 103 neurological conditions involving the brain (38%), cranial nerves (36%), the meninges (3%), the spinal cord (10%) and the peripheral nerves (14%); no patient had concomitant central and peripheral nerve involvements. In 59 (69%) patients, neurological involvement preceded or was present at the time of diagnosis of the disease. According to the classification proposed by Stern et al., 11 (13%) were classified as a definite NS, 61 (72%) as a probable NS and the remaining 13 (15%) as a possible NS. In comparison with the systemic phenotype of patients without NS, patients with CNS involvement presented a lower frequency of thoracic involvement (82% vs 93%, q = 0.018), a higher frequency of ocular (27% vs 10%, q < 0.001) and salivary gland (15% vs 4%, q = 0.002) WASOG involvements. In contrast, patients with PNS involvement showed a higher frequency of liver involvement (36% vs 12%, p = 0.02) in comparison with patients without NS. Neurosarcoidosis was identified in 5.5% of patients. CNS involvement prevails significantly over PNS involvement, and both conditions do not overlap in any patient. The systemic phenotype associated to each involvement was clearly differentiated, and can be helpful not only in the early identification of neurological involvement, but also in the systemic evaluation of patients diagnosed with neurosarcoidosis.
Behçet's Disease (BD) is a rare, chronic and recurrent inflammatory multisystemic condition of unknown origin that can affect any tissue. The vascular system is involved in 5-40% of cases of BD, ...including venous and arterial beds and it has a relapsing course. Budd-Chiari syndrome (BCS) is a rare complication of BD with a frequency of < 5% among patients with vascular involvement and is more frequent in men (89.5%). Two clinical presentation groups of BCS related to BD have been described: the "symptomatic" form and the "silent" form. We present a case of BD in a young woman presented as symptomatic severe BCS with rapid progression of coagulopathy reaching a spontaneous INR of 1.74 and increased ascites by ultrasound control. BD was confirmed through clinical history. The patient was treated with a high-dose pulse of corticosteroids and cyclophosphamide with a strikingly favorable response in the first forty-eight hours. Although several studies have demonstrated a survival improvement with the use of transjugular intrahepatic portosystemic shunt in patients with severe BCS, it was discarded due to the lack of evidence of this procedure in patients with BD and the fact that it could trigger a vascular pathergy phenomenon. Vascular BD should be suspected in recurrent venous and/or arterial thrombosis since it is associated with high morbidity and mortality. Immunosuppressive treatment is critical for the management of vascular involvement in BD. However, the role of anticoagulation is debatable. We suggest an algorithm for the management of BCS associated with BD.
Behçet syndrome (BS) is a unique type of vasculitis that affects veins and arteries of all sizes, leading to recurrent vascular events, mostly venous thrombosis. The prevalence of venous ...thromboembolism in BS patients ranges between 15 and 40%. Thrombosis is usually an early manifestation leading to diagnosis of BS in up to 40% of patients. BS is per se a model of inflammation-induced thrombosis. The primary autoimmune response activates lymphocytes that in turn produce a cytokine cascade that activates neutrophils, which modify the secondary structure of fibrinogen making it less susceptible to plasmin-induced lysis. This leads to endothelial dysfunction, platelet activation and overexpression of tissue factor leading to inflammatory thrombi, usually attached to the wall. The pathogenesis of thrombosis is especially relevant to direct the specific treatment, that is based on immunosuppression rather than anticoagulation. Superficial vein thrombosis (SVT) and deep vein thrombosis (DVT) are the most common form of thrombosis in BS, but thrombosis in atypical sites (cava vein, suprahepatic veins, intracardiac thrombus) and arterial involvement can also occur. We assessed the latest update of the European League Against Rheumatism recommendations for the management of BS. Vascular Behçet treatment is usually based of immunosuppressants, and the role of anticoagulation remains controversial. The use of interventional and surgical procedures should be carefully evaluated, due to the risk of triggering a vascular pathergy phenomenon.
Prevalence of neurological involvement in Behçet's disease (BD) varies between studies and is associated with high morbimortality. Neurological involvement is classified as parenchymal and ...non-parenchymal involvement (vascular involvement). Treatment of neurological involvement consists of immunosuppressants. Evidence regarding presentation and outcomes in neuro-Behçet's (NB) is scarce.
Observational retrospective study that included consecutive patients diagnosed with BD between 2006 and 2019 in a tertiary hospital of Madrid.
Fifty-seven patients were included with a mean follow-up of 7.83 (±6.98) years. The mean age at diagnosis of BD and first NB episode was 34.7 (± 12.1) and 31.03 (±9.9) years, respectively. Fifty-six NB episodes were registered in 25 patients. Fever (OR 3.39, CI 95% 1.14-10.15) and pseudofolliculitis (OR 4.06, CI 95% 1.11-14.80) were associated to NB. Trigeminal neuralgia was found in 16% (4/25) of patients.
The prevalence of NB in patients with BD was higher than previously described. Fever and pseudofolliculitis were more frequently associated with NB, with low mortality during follow-up.
Almost one third of patients with Behçet's syndrome (BS) display vascular involvement. However, data regarding the prevalence and management of venous thromboembolism (VTE) in BS are scanty. We ...assessed the differential characteristics between patients with and without VTE and the factors associated with VTE incidence. A case–control study in a cohort of patients with BS was performed. 57 patients were included (56.1% women) with a mean follow-up of 10.56 (± 10.7) years. Mean age at diagnosis of BS and diagnosis of the first VTE episode was 34.7 (± 12.1) and 31.2 (± 8.9) years, respectively. Erythema nodosum (OR 4.6, CI 95% 1.2–18.1) and fever (OR 8.2, CI 95% 1.6–42.1) were associated with a higher risk of VTE. 26 episodes of VTE were registered in 12/57 (21%) patients. 83.3% of patients were not diagnosed with BS when the first episode of VTE occurred and, among them, the episode of VTE led to the diagnosis of BS in 40% of cases. Half of patients had at least one VTE recurrence. The absence of immunosuppressive treatment was associated with a higher risk of developing a first episode of VTE (OR 20 CI 95% 19.2–166.6). All patients were treated with anticoagulation and 75% were treated with immunosuppressants after the first VTE event. The diagnosis of VTE usually precedes that of BS, with a high frequency of VTE recurrence. Erythema nodosum and fever were associated with a higher risk of VTE, while the immunosuppressants showed a protective role for the development of VTE.
Prevalence of neurological involvement in Behçet's disease (BD) varies between studies and is associated with high morbimortality. Neurological involvement is classified as parenchymal and ...non-parenchymal involvement (vascular involvement). Treatment of neurological involvement consists of immunosuppressants. Evidence regarding presentation and outcomes in neuro-Behçet's (NB) is scarce.
Observational retrospective study that included consecutive patients diagnosed with BD between 2006 and 2019 in a tertiary hospital of Madrid.
Fifty-seven patients were included with a mean follow-up of 7.83 (±6.98) years. The mean age at diagnosis of BD and first NB episode was 34.7 (±12.1) and 31.03 (±9.9) years, respectively. Fifty-six NB episodes were registered in 25 patients. Fever (OR 3.39, 95% CI: 1.14–10.15) and pseudofolliculitis (OR 4.06, 95% CI: 1.11–14.80) were associated to NB. Trigeminal neuralgia was found in 16% (4/25) of patients.
The prevalence of NB in patients with BD was higher than previously described. Fever and pseudofolliculitis were more frequently associated with NB, with low mortality during follow-up.
Las manifestaciones neurológicas en la enfermedad de Behçet (EB) presentan una prevalencia variable en la literatura y asocian una mayor morbimortalidad. Se distingue la afectación parenquimatosa y la no parenquimatosa (vascular). El tratamiento de estas manifestaciones se basa en la inmunosupresión. La evidencia sobre la presentación y evolución del neurobehçet (NB) es escasa.
Estudio observacional retrospectivo que incluyó a pacientes consecutivos con diagnóstico de EB entre 2006 y 2019 en un hospital terciario de Madrid.
Se incluyó a 57 pacientes con EB, con un seguimiento medio de 7,83 (±6,98) años. La edad media al diagnóstico de EB y del primer episodio de NB fue 34,7 (±12,1) años y 31 (±9,9) años, respectivamente. Se registraron 56 episodios de NB en 25 pacientes. La fiebre (OR 3,39; IC 95%: 1,14-10,15) y la pseudofoliculitis (OR 4,06; IC 95%: 1,11-14,80) se asociaron a NB. La neuralgia del trigémino se encontró en el 16% (4/25) de los pacientes.
La prevalencia de NB fue mayor que la descrita previamente. La fiebre y la pseudofoliculitis se asociaron a NB y la mortalidad fue baja durante el seguimiento.
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