No abstract

BackgroundVascular involvement especially in young males and is one of the major causes of mortality and morbidity. Lower extremity deep vein thrombosis (DVT) is the most frequent form of vascular involvement. Chronic post-thrombotic syndrome develops in up to one-half of patients with deep venous thrombosis. Patients with post-thrombotic syndrome have substantially impaired quality of life.ObjectivesThere is no data about post-thrombotic syndrome in deep venous thrombosis associated with Behçet's Disease. In this study,we aimed to evaluate venous disease spesific quality of life in patients with Vascular Behçet's Disease (VBD).MethodsIn this study, 62 patients (Male/Female: 47/15, mean age: 38.6±9.2 years) with VBD, who were regularly followed in Marmara University Behçet clinic, 29 patients (Male/Female: 20/9, mean age: 41.6±11.8 years) with DVT associated with non-BD reasons, who were followed in Hematology and Vascular Surgery Department, were assessed. Venous Disability Score (VDS) and Venous Clinical Severity Score (VCSS) were used to evaluate the severity of venous insufficiency. Venous disease-specific quality of life (the primary outcome) was measured using the Turkish validated form of Venous Insufficiency Epidemiological and Economic Study Quality of Life/Symptom questionnaire (VEINES-QoL/Sym). After calculation, VEINES-QoL and VEINES-Sym sub-score were identified. Higher score shows better QoL. Short Form (SF-36) and Behçet Syndrom Activity Score (BSAS) questionnaire were also fullfilled to patients.ResultsPhysical and mental component of SF-36 were similar between VBD and non-BD group. VCSS and VDS were significantly worse in non-BD group compared to VBD. VEINES-QoL and VEINES-Sym were significantly better in VBD group (Table 1). Mean duration after DVT was 6.7±5 years in VBD, 1.6±1 years in non-BD group. There was no correlation between VEINES-QoL, VEINES-Sym score and duration after DVT, daily sitting and standing time. But VEINES-QoL and VEINES-Sym negatively correlated with VCSS (respectively; r: -0.367, p<0.001, r:-0.384, p<0.001) and VDS (respectively, r: -0.288, p=0.007, r:-0.265, p=0.013). VEINES-Sym and VEINES-QoL also significantly correlated with BSAS (respectively, r: -0.526, p<0.001 ve r:-0.496, p<0.001).Table 1.Quality of life parameters in patients with Deep Vein Thrombosis associated with both Behçet's disease and non-Behçet reasonsNon-Behçet group (n=29)Behçet group (n=62)p valueVCSS6.5±4.54.2±4.60.031VDS1.6±0.50.9±0.60.000MCS40.8±10.244.9±9.50,071PCS40.3±9.343.1±10.50.222VEINES-QoL73.3±17.987.9±16.90.000VEINES-Sym32.3±9.839.3±9.20.001VCSS, Venous Clinical Severity Score; VDS, Venous Disability Score; MCS, Mental component of SF-36; PCS, Physical component of SF-36; VEINES-QoL/Sym, Venous Insufficiency Epidemiological and Economic Study Quality of Life/Symptom questionnaire.ConclusionsIn our study, we found that venous spesific QoL and symptoms worsen together with increasing VDS and VCSS score. These results suggest that controlling of disease activity increase the OoL as well as pre...

No abstract

bortezomib, seven patients (53.8%) to lenalidomide, and eight patients (61.5%) to last treatment. At relapse, two (15.4%) had high LDH, eight (61.5%) impaired renal function, and three (23.1%) extramedullary disease. Treatment was dara-lenalidomide-dex in six (46.2%), and dara-pomalidomide-dex in seven (53.8%). Results: Responses after four cycles included CR in 5 patients (38.5%), VGPR in five patients (38.5%), and PR in three patients (23.1%). After a median of four dara infusions (range: 3-10), the best responses included CR in seven patients (53.8%), nCR in two patients (15.4%), VGPR in two patients (15.4%), and PR in two patients (15.4%). Median time to VGPR was one month. At 10 months, the OS was 90%, and PFS 54.7%. Three patients progressed, one of whom died of ruptured hepatic plasmacytoma. The most frequent toxicity was haematological especially neutropenia

BackgroundMajor organ involvement is one of the main causes of mortality and morbidity in Behçet’s Disease (BD) [1]. However, the prognosis and predictors of major organ involvement are insufficiently studied.ObjectivesWe aimed to follow young, male BD patients with only mucocutaneous symptoms who have the highest risk for new major organ involvement prospectively.MethodsThirty-six male patients with BD were included in the study. Patients with BD were assessed prospectively at 3-6 months intervals and in any urgent visits. New major organ involvements and reasons for immunosuppressive (IS) need were assessed during prospective follow-up.ResultsAt baseline, the mean disease duration was 3.3 years. All patients were under colchicine treatment. The mean follow-up duration was 90.7 months. Overall, 13 (36.1%) patients needed IS therapy during follow-up. The reason for IS need was major organ involvement in 5 (13.9%), refractory mucocutaneous involvement in 7 (19.4%), and articular involvement in 1 (2.8%)(Table 1, Figure 1). Major organ involvement was vascular in 3 patients, ocular in 1 patient, and ocular and vascular in 1 patient. In 8 of these 13 patients, step-up treatment was needed in ISs due to refractory disease or relapse.ConclusionOur study demonstrated a lower incidence of major vascular events in male BD patients during prospective follow-up compared to retrospective cohorts in the literature. Our results showed that refractory mucocutaneous involvement is a more frequent reason for IS need in BD than major organ involvement during prospective follow-up.Reference[1]Alibaz-Oner F, Direskeneli H. Management of vascular Behçet’s disease. Int J Rheum Dis. 2019;22 Suppl 1:105-8.Table 1.Clinical characteristics of patients with immunosuppressive treatment during follow-upReason for IS useAge at DiagnosisDisease duration when IS startedIS agentPatient 1Pulmonary aneurysm351 yearAzathioprinePatient 2Refractory OU255 yearsCyclosporinePatient 3Deep venous thrombosis3810 yearsAzathioprinePatient 4Uveitis205 yearsAzathioprinePatient 5Refractory OU287 yearsAzathioprinePatient 6Refractory OU236 yearsCyclosporinePatient 7Refractory EN351 yearsAzathioprinePatient 8Deep venous thrombosis231 yearsAzathioprinePatient 9Arthritis287 yearsMethotrexatePatient 10Refractory OU2913 yearsAzathioprinePatient 11Refractory EN+ Articular Involvement235 yearsAzathioprinePatient 12Superficial Thrombophlebitis292 yearsAzathioprinePatient 13Refractory OU283 yearsAzathioprineIS: Immunosuppressive, OU: Oral ulcer, EN: erythema nodosumFigure 1.The reasons for immunosupressive need in Behçet patients during follow-up (n)AcknowledgementsI have no acknowledgments to declare.Disclosure of InterestsNone Declared.