scholarly journals POS0324 SMOKING HISTORY AND SCLERODERMA CLINICAL TRIALS CONSORTIUM DAMAGE INDEX (SCTC-DI) AS INDEPENDENT PREDICTORS OF MORTALITY IN SYSTEMIC SCLEROSIS IN A SINGLE-CENTRE ITALIAN COHORT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 389.1-389
Author(s):  
M. Breda ◽  
M. G. Lazzaroni ◽  
F. Franceschini ◽  
P. Airò

Background:Systemic Sclerosis (SSc) is characterized by increased mortality and organ damage accrual. A composite SSc Damage Index was recently developed by the Scleroderma Clinical Trials Consortium (SCTC-DI) and was demonstrated as a predictor of mortality both in the Australian derivation cohort and in the Canadian validation cohort (1). Several independent predictors of mortality in SSc have been reported, but only limited data are available on the role of smoking.Objectives:To evaluate smoking history and SCTC-DI as independent predictors of mortality in SSc in a single centre Italian cohort.Methods:A retrospective analysis was performed on patients prospectively followed in our centre from 1989 to 2019, with at least 2 evaluations and/or cause of death available. Organ damage was evaluated through the SCTC-DI (0-55 scale; severe damage>12), while comorbidities through the Charlson Comorbidity Index (CCI). Survival analysis was performed with Kaplan-Meier curves and with Log-rank test to compare different subsets. Cox-regression analysis was performed to identify baseline independent predictors of mortality.Results:648 SSc patients were 99% Caucasian, 90% female and had a median age at diagnosis of 55.5 years (IQR: 45.0-65.6); 19% had diffuse cutaneous involvement. ACA was positive in 52%; anti-TopoI in 22% and anti-RNA Polymerase III in 5%.Median SCTC-DI at diagnosis was 2 (0-4) (n=560); ever smokers were 27%. During the follow-up 240 patients died after a median period of 10.2 years (4.9-16.8). The cause of death was related to SSc in 41% (n=65; most frequent causes pulmonary arterial hypertension (n=35) and interstitial lung disease (n=30)) and to other diseases in 40% (n=95; most frequent cause cancer (n=40)), while was indeterminate in 19%.Overall survival at 5, 10, 15, 20 years was 87.8% (SE 1.3%), 75.6% (1.9), 63.8% (2.3), 47.7% (2.8), respectively and was higher in females vs. males (p<0.0001) and in ACA+ vs ACA- (p=0.005), but did not differ between cutaneous subsets. 111 patients were lost at follow-up (17%).In the Cox-regression analysis smoking history and severe organ damage at diagnosis (score>12) were identified as independent predictors of death, while age at diagnosis, female gender, diffuse cutaneous subsets, ACA positivity and CCI were not (Table 1).Conclusion:Smoking history and severe organ damage were independent predictors of death in a large Italian single centre SSc cohort. SCTC-DI was confirmed as a useful tool to predict mortality at every timepointReferences:[1]Ferdowsi N, et al. Ann Rheum Dis. 2019;78:807-16.Disclosure of Interests:None declared

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
K. P. Thompson ◽  
◽  
J. Nelson ◽  
H. Kim ◽  
L. Pawlikowska ◽  
...  

Abstract Background Retrospective questionnaire and healthcare administrative data suggest reduced life expectancy in untreated hereditary hemorrhagic telangiectasia (HHT). Prospective data suggests similar mortality, to the general population, in Denmark’s centre-treated HHT patients. However, clinical phenotypes vary widely in HHT, likely affecting mortality. We aimed to measure predictors of mortality among centre-treated HHT patients. HHT patients were recruited at 14 HHT centres of the Brain Vascular Malformation Consortium (BVMC) since 2010 and followed annually. Vital status, organ vascular malformations (VMs) and clinical symptoms data were collected at baseline and during follow-up (N = 1286). We tested whether organ VMs, HHT symptoms and HHT genes were associated with increased mortality using Cox regression analysis, adjusting for patient age, sex, and smoking status. Results 59 deaths occurred over average follow-up time of 3.4 years (max 8.6 years). A history of anemia was associated with increased mortality (HR = 2.93, 95% CI 1.37–6.26, p = 0.006), as were gastro-intestinal (GI) bleeding (HR = 2.63, 95% CI 1.46–4.74, p = 0.001), and symptomatic liver VMs (HR = 2.10, 95% CI 1.15–3.84, p = 0.015). Brain VMs and pulmonary arteriovenous malformations (AVMs) were not associated with mortality (p > 0.05). Patients with SMAD4 mutation had significantly higher mortality (HR = 18.36, 95% CI 5.60–60.20, p < 0.001) compared to patients with ACVRL1 or ENG mutation, but this estimate is imprecise given the rarity of SMAD4 patients (n = 33, 4 deaths). Conclusions Chronic GI bleeding, anemia and symptomatic liver VMs are associated with increased mortality in HHT patients, independent of age, and in keeping with the limited treatment options for these aspects of HHT. Conversely, mortality does not appear to be associated with pulmonary AVMs or brain VMs, for which patients are routinely screened and treated preventatively at HHT Centres. This demonstrates the need for development of new therapies to treat chronic anemia, GI bleeding, and symptomatic liver VMs in order to reduce mortality among HHT patients.


Rheumatology ◽  
2020 ◽  
Author(s):  
Jens Rathmann ◽  
David Jayne ◽  
Mårten Segelmark ◽  
Göran Jönsson ◽  
Aladdin J Mohammad

Abstract Objective To determine the incidence rate, predictors and outcome of severe infections in a population-based cohort of ANCA-associated vasculitis (AAV). Methods The study included 325 cases of AAV (152 female) diagnosed from 1997 through 2016 from a defined geographic area in Sweden. All severe infection events (requiring hospitalization and treatment with intravenous antimicrobials) were identified. The Birmingham vasculitis activity score (BVAS) was used to evaluate disease activity, and organ damage was assessed using the vasculitis damage index (VDI). Patients were followed from time of AAV diagnosis to death or December 2017. Results A total of 129 (40%) patients suffered at least one severe infection. In 2307 person-years (PY) of follow-up, 210 severe infections were diagnosed. The incidence rate of severe infections was 9.1/100 PY and was highest during the first year following AAV diagnosis at 22.1/100 PY (P &lt; 0.001). Pneumonia, sepsis and urinary tract infection were the most common infections. Opportunistic infections constituted only 6% of all severe infections. In Cox regression analysis age and BVAS at diagnosis were the only factors independently predicting severe infection [hazard ratio: 1.54 (P &lt; 0.001) and 1.27 (P = 0.001), respectively]. Severe infection was associated with poorer prognosis with respect to median VDI score 12 months post-AAV diagnosis, renal survival and mortality. Severe infections were the cause of death in 32 patients (22% of all deaths). Conclusion . Severe infection is a common problem in AAV, with the most important prognostic factors being older age and high disease activity at diagnosis. Severe infections are associated with permanent organ damage and high mortality.


Author(s):  
O.A. Radaeva ◽  
A.S. Simbirtsev

Essential hypertension (EH) is one of the most common modifiable risk factors for cardiovascular diseases and death. The aim of this study was to investigate a correlation between the levels of some cytokines (interleukins, adhesion molecules, tumor necrosis and growth factors, etc.) in the peripheral blood of patients with stage II EH and the rate of complications (myocardial infarction, acute cerebrovascular events, and transient ischemic attacks) occurring in a 5-year follow-up period. Twenty-eight cytokines were measured using ELISA, including IL1β, IL1α, IL1ra, IL18, IL18BP, IL37, IL6, sIL6r, LIF, sLIFr, IGF-1, IGFBP-1, TNFα, sTNF-RI, sVCAM-1, IL17, IL2, IL4, IL10, TGF-β1, IL8, CX3CL1, CXCL10, INFγ, M-CSF, IL34, VEGF-A, and erythropoietin, and a few vasoactive peptides, including NО, iNOS, eNOS, ADMA, SDMA, Nt-proСNP, and Nt-proBNP, in the peripheral blood samples of 200 patients with stage II EH who had been suffering from this condition for 10 to 14 years and were receiving comparable therapies to bring their blood pressure down. The patients were followed up for 5 years to keep track of complications. The retrospective analysis revealed that the group of patients who developed complications during the 5-year follow-up period exhibited a decline in the levels of IL1ra (р < 0.001) and IL10 (р < 0.001) and a rise in IL1β (р < 0.001), TNFα (р < 0.001) and M-CSF (р < 0.001) in comparison with the group of those who did not develop any complications. The multivariate Cox regression analysis was applied to the following parameters: IL1β > 18.8 pg/ml; IL1ra < 511 pg/ml; IL6 > 23.8 pg/ml; IL10 < 26.3 pg/ml; 389 pg/ml < M-CSF < 453 pg/ml; ADMA > 0.86 μmol/L; total cholesterol > 4.9 mmol/L; LDL> 3.0 mmol/L; HDL in men < 1.0 mmol/L; HDL in women < 1.2 mmol/L. The analysis revealed that M-CSF in the range from 389 to 453 pg/ml (р < 0.001) and LDL above 3.0 mmol/L (р < 0.01) correlated with an increase in the risk for end-organ damage in stage II EH. Changes in the cytokine levels can be regarded as a predictor of myocardial and cerebral damage in patients with stage II EH. Measurement of peripheral blood M-CSF can be included into the classic risk assessment schemes for the cardiovascular complications in the studied cohort of patients.


2021 ◽  
Vol 12 ◽  
Author(s):  
Xianqiu Chen ◽  
Jian Guo ◽  
Dong Yu ◽  
Bing Jie ◽  
Ying Zhou

Background: Progressive fibrosing interstitial lung disease (PF-ILD) and idiopathic pulmonary fibrosis (IPF) share similar progression phenotype but with different pathophysiological mechanism. The purpose of this study was to assess clinical characteristics and outcomes of patients with PF-ILD in a single-center cohort.Methods: Patients with PF-ILD treated in Shanghai Pulmonary Hospital from Jan. 2013 to Dec. 2014 were retrospectively analyzed. Baseline characteristics and clinical outcomes were collected for survival analysis to identifying clinical predictors of mortality.Results: Among 608 patients with ILD, 132 patients met the diagnostic criteria for PF-ILD. In this single-center cohort, there were 51 (38.6%) cases with connective tissue disease-associated interstitial lung disease (CTD-ILD) and 45 (34.1%) with unclassifiable ILDs. During follow-up, 83 patients (62.9%) either died (N = 79, 59.8%) or underwent lung transplantations (N = 4, 3.0%) with a median duration follow-up time of 53.7 months. Kaplan-Meier survival curves revealed that the 1, 3 and 5-years survival of PF-ILD were 90.9, 58.8 and 48.1%, respectively. In addition, the prognosis of patients with PF-ILD was similar to those with IPF, while it was worse than non-PF-ILD ones. Multivariate Cox regression analysis demonstrated that high-resolution computed tomography (HRCT) scores (HR 1.684, 95% CI 1.017–2.788, p = 0.043) and systolic pulmonary artery pressure (SPAP) &gt; 36.5 mmHg (HR 3.619, 95%CI 1.170–11.194, p = 0.026) were independent risk factors for the mortality of PF-ILD.Conclusion: Extent of fibrotic changes on HRCT and pulmonary hypertension were predictors of mortality in patients with PF-ILD.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 166.2-166
Author(s):  
M. G. Lazzaroni ◽  
M. Breda ◽  
F. Franceschini ◽  
P. Airò

Background:Systemic Sclerosis (SSc) is characterized by increased mortality and organ damage accrual. A composite SSc Damage Index was recently developed by the Scleroderma Clinical Trials Consortium (SCTC-DI) and was demonstrated as a predictor of mortality both in the Australian derivation cohort and in the Canadian validation cohort.Objectives:To evaluate in a single centre cohort of SSc patients with 10-years follow-up: (1) the evolution of organ damage over time; (2) factors associated with the development and accrual of organ damage.Methods:A retrospective analysis was performed on patients prospectively followed in our centre from 1989 to 2019. Organ damage was evaluated with SCTC-DI (0-55 scale; moderate damage >5, severe damage>12) and comorbidities with Charlson Comorbidity Index (CCI, which includes the age of the patient). Patients were included when a follow-up of at least 10 years was available together with SCTC-DI at the diagnosis (baseline, T0), 1 year (T1), 5 years (T5) and 10 years (T10) after the diagnosis. Univariable and multivariable analysis (logistic regression) were performed when appropriated.Results:253 SSc patients were included (female 93%; Caucasians: 99%; median age at diagnosis: 52 years (IQR: 43-60); diffuse cutaneous subset: 15%; anti-centromere (ACA)+ 55%; anti-Topoisomerase 1 + 20%; anti-RNA polymerase III+: 4%; ever smokers: 28%). Median interval between the first SSc symptom other than Raynaud’s phenomenon and the diagnosis was 1 year. SCTC-DI progressively increased from diagnosis to T10 (p<0.0001; Kruskal-Wallis test).Moderate damage (score:6-12) was observed in 22 patients at T0 (8.7%), in 30 at T1 (11.9%), in 45 at T5 (17.7%) and in 73 at T10 (28.9%). None of the patients had severe damage (score:13-55) at T0 and T1, while it was present in 6 at T5 (2.4%) and in 13 at T10 (5.1%).At T0 no difference in SCTC-DI scores was observed when comparing different subgroups according to gender (female vs. male), disease subsets (diffuse vs. limited) and autoantibodies (ACA- vs. ACA+). At T1, SCTC-DI score was higher in patients with diffuse vs. limited cutaneous subset, and ACA- vs ACA+ (p<0.0001 for both).Multivariable analysis demonstrated that a moderate or severe organ damage (SCTC-DI score >5) at 5 years was positively associated with diffuse cutaneous involvement (p:0.009, OR 4.55, 1.46-14.1), SCTC-DI at T0 (p:0.015, OR 1.34, 1.06-1.70) and at T1 (p:<0.0001, OR 1.65, 1.30-2.07), and negatively associated with ACA+ (p:0.024, OR 0.32, 0.12-0.86), while CCI and male sex showed no association. At 10 years SCTC-DI>5 was associated with diffuse cutaneous involvement (p:0.013, OR 4.30, 1.36-13.7), SCTC-DI at T5 (p:<0.0001, OR 1.67, 1.38-2.01), while SCTC-DI at T0, CCI, male sex and ACA+ had no association.Among 253 patients, 90 (36%) died after >10 years of follow-up. In non-survivors, as compared to survivors, SCTC-DI score was significantly higher at the baseline (T0) and during the entire follow-up (p<0.0001 for every timepoint).Conclusion:At the end of 10-years follow-up (T10), 35% of patients in our cohort had moderate or severe organ damage (SCTC-DI score>5). Diffuse cutaneous involvement was associated with higher SCTC-DI scores at different time points (T5 and T10). Organ damage, quantified by SCTC-DI at different time points, was confirmed as a factor associated with mortality in patients who reached more than 10 years of follow-up.References:[1]Ferdowsi N, et al. Ann Rheum Dis. 2019;78:807-16.Disclosure of Interests:None declared


2015 ◽  
Vol 24 (3) ◽  
pp. 287-292 ◽  
Author(s):  
Petra A. Golovics ◽  
Laszlo Lakatos ◽  
Michael D. Mandel ◽  
Barbara D. Lovasz ◽  
Zsuzsanna Vegh ◽  
...  

Background & Aims: Limited data are available on the hospitalization rates in population-based studies. Since this is a very important outcome measure, the aim of this study was to analyze prospectively if early hospitalization is associated with the later disease course as well as to determine the prevalence and predictors of hospitalization and re-hospitalization in the population-based ulcerative colitis (UC) inception cohort in the Veszprem province database between 2000 and 2012. Methods: Data of 347 incident UC patients diagnosed between January 1, 2000 and December 31, 2010 were analyzed (M/F: 200/147, median age at diagnosis: 36, IQR: 26-50 years, follow-up duration: 7, IQR 4-10 years). Both in- and outpatient records were collected and comprehensively reviewed. Results: Probabilities of first UC-related hospitalization were 28.6%, 53.7% and 66.2% and of first re-hospitalization were 23.7%, 55.8% and 74.6% after 1-, 5- and 10- years of follow-up, respectively. Main UC-related causes for first hospitalization were diagnostic procedures (26.7%), disease activity (22.4%) or UC-related surgery (4.8%), but a significant percentage was unrelated to IBD (44.8%). In Kaplan-Meier and Cox-regression analysis disease extent at diagnosis (HR extensive: 1.79, p=0.02) or at last follow-up (HR: 1.56, p=0.001), need for steroids (HR: 1.98, p<0.001), azathioprine (HR: 1.55, p=0.038) and anti-TNF (HR: 2.28, p<0.001) were associated with the risk of UC-related hospitalization. Early hospitalization was not associated with a specific disease phenotype or outcome; however, 46.2% of all colectomies were performed in the year of diagnosis. Conclusion: Hospitalization and re-hospitalization rates were relatively high in this population-based UC cohort. Early hospitalization was not predictive for the later disease course.


2020 ◽  
Vol 17 (3) ◽  
pp. 218-223
Author(s):  
Haichao Wang ◽  
Li Gong ◽  
Xiaomei Xia ◽  
Qiong Dong ◽  
Aiping Jin ◽  
...  

Background: Depression and anxiety after stroke are common conditions that are likely to be neglected. Abnormal red blood cell (RBC) indices may be associated with neuropsychiatric disorders. However, the association of RBC indices with post-stroke depression (PSD) and poststroke anxiety (PSA) has not been sufficiently investigated. Methods: We aimed to investigate the trajectory of post-stroke depression and anxiety in our follow- up stroke clinic at 1, 3, and 6 months, and the association of RBC indices with these. One hundred and sixty-two patients with a new diagnosis of ischemic stroke were followed up at 1, 3, and 6 months, and underwent Patient Health Questionnaire-9 (PHQ-9) and the general anxiety disorder 7-item (GAD-7) questionnaire for evaluation of depression and anxiety, respectively. First, we used Kaplan-Meier analysis to investigate the accumulated incidences of post-stroke depression and post-stroke anxiety. Next, to explore the association of RBC indices with psychiatric disorders after an ischemic stroke attack, we adjusted for demographic and vascular risk factors using multivariate Cox regression analysis. Results: Of the 162 patients with new-onset of ischemic stroke, we found the accumulated incidence rates of PSD (1.2%, 17.9%, and 35.8%) and PSA (1.2%, 13.6%, and 15.4%) at 1, 3, and 6 months, respectively. The incident PSD and PSA increased 3 months after a stroke attack. Multivariate Cox regression analysis indicated independent positive associations between PSD risk and higher mean corpuscular volume (MCV) (OR=1.42, 95% CI=1.16-1.76), older age (OR=2.63, 95% CI=1.16-5.93), and a negative relationship between male sex (OR=0.95, 95% CI=0.91-0.99) and PSA. Conclusion: The risks of PSD and PSA increased substantially 3 months beyond stroke onset. Of the RBC indices, higher MCV, showed an independent positive association with PSD.


2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Cheng-Jui Lin ◽  
Chi-Feng Pan ◽  
Chih-Kuang Chuang ◽  
Fang-Ju Sun ◽  
Duen-Jen Wang ◽  
...  

Background/Aims. Previous studies have reported p-cresyl sulfate (PCS) was related to endothelial dysfunction and adverse clinical effect. We investigate the adverse effects of PCS on clinical outcomes in a chronic kidney disease (CKD) cohort study.Methods. 72 predialysis patients were enrolled from a single medical center. Serum biochemistry data and PCS were measured. The clinical outcomes including cardiovascular event, all-cause mortality, and dialysis event were recorded during a 3-year follow-up.Results. After adjusting other independent variables, multivariate Cox regression analysis showed age (HR: 1.12,P=0.01), cardiovascular disease history (HR: 6.28,P=0.02), and PCS (HR: 1.12,P=0.02) were independently associated with cardiovascular event; age (HR: 0.91,P<0.01), serum albumin (HR: 0.03,P<0.01), and PCS level (HR: 1.17,P<0.01) reached significant correlation with dialysis event. Kaplan-Meier analysis revealed that patients with higher serum p-cresyl sulfate (>6 mg/L) were significantly associated with cardiovascular and dialysis event (log rankP=0.03, log rankP<0.01, resp.).Conclusion. Our study shows serum PCS could be a valuable marker in predicting cardiovascular event and renal function progression in CKD patients without dialysis.


Author(s):  
Mustafa Umut Somuncu ◽  
Belma Kalayci ◽  
Ahmet Avci ◽  
Tunahan Akgun ◽  
Huseyin Karakurt ◽  
...  

AbstractBackgroundThe increase in soluble suppression of tumorigenicity 2 (sST2) both in the diagnosis and prognosis of heart failure is well established; however, existing data regarding sST2 values as the prognostic marker after myocardial infarction (MI) are limited and have been conflicting. This study aimed to assess the clinical significance of sST2 in predicting 1-year adverse cardiovascular (CV) events in MI patients.Materials and methodsIn this prospective study, 380 MI patients were included. Participants were grouped into low sST2 (n = 264, mean age: 60.0 ± 12.1 years) and high sST2 groups (n = 116, mean age: 60.5 ± 11.6 years), and all study populations were followed up for major adverse cardiovascular events (MACE) which are composed of CV mortality, target vessel revascularization (TVR), non-fatal reinfarction, stroke and heart failure.ResultsDuring a 12-month follow-up, 68 (17.8%) patients had MACE. CV mortality and heart failure were significantly higher in the high sST2 group compared to the low sST2 group (15.5% vs. 4.9%, p = 0.001 and 8.6% vs. 3.4% p = 0.032, respectively). Multivariate Cox regression analysis concluded that high serum sST2 independently predicted 1-year CV mortality [hazard ratio (HR) 2.263, 95% confidence interval (CI) 1.124–4.557, p = 0.022)]. Besides, older age, Killip class >1, left anterior descending (LAD) as the culprit artery and lower systolic blood pressure were the other independent risk factors for 1-year CV mortality.ConclusionsHigh sST2 levels are an important predictor of MACE, including CV mortality and heart failure in a 1-year follow-up period in MI patients.


Author(s):  
Julia Götte ◽  
Armin Zittermann ◽  
Kavous Hakim-Meibodi ◽  
Masatoshi Hata ◽  
Rene Schramm ◽  
...  

Abstract Background Long-term data on patients over 75 years undergoing mitral valve (MV) repair are scarce. At our high-volume institution, we, therefore, aimed to evaluate mortality, stroke risk, and reoperation rates in these patients. Methods We investigated clinical outcomes in 372 patients undergoing MV repair with (n = 115) or without (n = 257) tricuspid valve repair. The primary endpoint was the probability of survival up to a maximum follow-up of 9 years. Secondary clinical endpoints were stroke and reoperation of the MV during follow-up. Univariate and multivariable Cox regression analysis was performed to assess independent predictors of mortality. Mortality was also compared with the age- and sex-adjusted general population. Results During a median follow-up period of 37 months (range: 0.1–108 months), 90 patients died. The following parameters were independently associated with mortality: double valve repair (hazard ratio, confidence interval [HR, 95% CI]: 2.15, 1.37–3.36), advanced age (HR: 1.07, CI: 1.01–1.14 per year), diabetes (HR: 1.97, CI: 1.13–3.43), preoperative New York Heart Association (NYHA) functional class (HR: 1.41, CI: 1.01–1.97 per class), and operative creatininemax levels (HR: 1.32, CI: 1.13–1.55 per mg/dL). The risk of stroke in the isolated MV and double valve repair groups at postoperative year 5 was 5.0 and 4.1%, respectively (p = 0.65). The corresponding values for the risk of reoperation were 4.0 and 7.0%, respectively (p = 0.36). Nine-year survival was comparable with the general population (53.2 vs. 53.1%). Conclusion Various independent risk factors for mortality in elderly MV repair patients could be identified, but overall survival rates were similar to those of the general population. Consequently, our data indicates that repairing the MV in elderly patients represents a suitable and safe surgical approach.


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