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...
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.
Results: Median age of patient population was 53 (28-90) years. Fifty-four (44.6%) was female. Eighty-three patients (81.4%) had Sokal low-intermediate disease. Most of the patients had good ECOG performance status (<2: 96%) and low hematopoietic cell transplantation-comorbidity index score (<3: 90.8%). Median duration of TKIs was 31 (3-154) months. Ninety-eight patients (86.7%) had major molecular response at the time of questionnaire. Patients receiving different TKIs had comparable quality of life and treatment-related symptom burden scores (table and figure). Summary/Conclusion:First and second generation TKIs as well as generic imatinib have similar impact on quality of life. Although these molecules have different safety profiles, severity of treatment-related adverse events are generally mild.
Background: Periodic Upper Gastrointestinal Endoscopy (UGIE) for Oesophageal Varices (OVs) is recommended in all compensated cirrhotic patients. The prevalence of early stage cirrhosis is on the rise with the contribution of non-invasive models for liver fibrosis. The healthcare system needs to focus on cost-effective surveillance strategies re-channelling its efforts for high-risk OVs. This study evaluated whether non-invasive means for prediction of high-risk OVs could limit UGIEs among the the Child-Pugh class A cirrhosis population. Materials and Methods: Endoscopic, clinical, and laboratory data were retrieved from medical records. Shear-Wave Elastography (SWE) and ultrasound spleen measurements, Platelet Count/Spleen Diameter (PC/SD) ratio and Child-Pugh score calculations were performed. Results: Successful elasticity acquisitions were performed in 99 cirrhosis patients. Child-Pugh score was A in 67.2% cases. The PC/SD
BackgroundBehçet's disease (BD) is a systemic disease characterized by oral aphthosis, genital ulcers, ocular lesions and systemic involvement including major vessels. Vascular Behçet's disease (VBD) is observed in up to 40% of BD. It is one of the major causes ofmorbidity and mortality in BD. Deep venous thrombosis (DVT) is the most common form of vascular involvement in Behcet's disease (BD). Post-thrombotic syndrome (PTS) develops in up to one-half of patients with DVT and is associated with impaired quality of life (QoL). There is no data on severity of DVT related PTS and its impact on quality of life in patients with Vascular Behçet's Disease (VBD).ObjectivesWe aimed to evaluate frequency of PTS and venous disease spesific QoL in VBD and to compare it with patients having DVT associated with non-Behçet's disease (NBD) causes.MethodsThis study included 50 patients (Male/Female: 41/9) with VBD and 31 age and gender-matched individuals (Male/Female: 22/9), with DVT associated with non-BD causes. Villalta scale was used to assess of PTS. Venous Disability Score (VDS) and Venous Clinical Severity Score (VCSS) were used for the assessment of venous disease. Venous disease-specific QoL was measured through Venous Insufficiency Epidemiological and Economic Study Quality of Life/Symptom questionnaire (VEINES-QoL/Sym). Behçet Syndrom Activity Score (BSAS) questionnaire was used to assess disease activity.ResultsWe observed significantly lower risk of PTS together with significantly better VEINES-QoL, VEINES-Sym and VDS in VBD group compared to NBD group. When VBD patients with PTS were compared to patients without PTS; VEINES-QoL, VEINES-Sym VCSS were significantly worse. BSAS was also significantly higher in patients with PTS. An inverse correlation was found between VEINES-QoL and BSAS in multivariate analysis. There were no differences between anticoagulant users and non-users regarding the presence of PTS and scores of all venous assessment tools.Table 1.Venous assessment and quality of life parameters in study groupsVascular Behçet disease (n=50)Non-Behçet group (n=31)P valueVEINES-QoL85.6±17.174.9±18.60.01VEINES-Sym37.7±9.433.1±10.10.04VCSS5.2±5.16.2±4.50.346CEAP2.4±1.72.3±1.50.747VDS1.1±0.61.5±0.50.002SF-36 MCS45.3±9.641.9±10.80.163SF-36 PCS42.1±10.741.2±9.60.726VEINES-QoL/Sym: Venous Insufficiency Epidemiological and Economic Study Quality of Life/Symphtom questionnaire, VCSS: Venous Clinical Severity Score, CEAP: Clinical, Etiologic, Anatomic, Pathophysiologic) classification, VDS: Venous Disability Score MCS: Mental component of SF-36, PCS: Physical component of SF-36.ConclusionsWe found lower PTS risk and better venous disease spesific QoL, symptom severity and venous disability scores in VBD compared to NBD group. Venous disease spesific QoL negatively correlated with disease activity. Effective control of vascular inflammation through treatment in VBD might explain lower PTS and better venous QoL outcomes in VBD patients. Our results suggest that successful control of disease activity might decrease devel...
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