scholarly journals Myositis-specific autoantibodies and their clinical associations in Idiopathic Inflammatory Myopathies

2019 ◽  
Author(s):  
Victor Tak Lung Wong ◽  
Ho SO ◽  
Ronald Man Lung Yip

Abstract Background The aims of the study were to investigate the prevalence of myositis specific autoantibodies (MSAs) and their associated complications in a cohort of patients with idiopathic inflammatory myopathies (IIMs). Methods A total of 201 consecutive patients with IIMs being followed up in the Rheumatology clinics of the participating regional hospitals in Hong Kong from July 2016 to January 2018 were recruited. Clinical characteristics, treatment history and disease complications were documented. Immunoblot assay was used to detect the MSAs.Results Out of the 201 patients, at least one MSA was found in 63.2% of patients. The most common MSAs were the anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) and the anti-transcriptional intermediary factor 1-gamma antibody (anti-TIF1-γ Ab) (both 13.9%), followed by anti-Jo-1 antibody (12.4%). Anti-MDA5 Ab was present exclusively in dermatomyositis (DM) and was strongly associated with digital ulcers, the clinically amyopathic phenotype and rapidly progressive interstitial lung disease (RP-ILD). Anti-TIF1γ Ab was strongly associated with refractory rash and malignancy. Multivariate analysis showed that the independent risk factors of RP-ILD included anti-MDA5 Ab (OR 14.5, p=0.001), clinically amyopathic DM (OR 13.9, p=0.015) and history of pulmonary tuberculosis (OR 12.2, p=0.026). Cox regression analysis showed that the independent predictors of malignancy included anti-TIF1γ Ab (HR 3.55, p=0.002), DM (HR 3.82, p=0.009) and family history of cancer (HR 3.40, p=0.038). Conclusions MSA testing enables dividing of patients with IIMs into phenotypically homogenous subgroups and prediction of potentially life-threatening complications.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 708.1-708
Author(s):  
J. S. Lee ◽  
S. H. Nam ◽  
S. J. Choi ◽  
W. J. Seo ◽  
S. Hong ◽  
...  

Background:Several studies have been conducted on factors associated with mortality in idiopathic inflammatory myopathies (IIM), but few studies have assessed prognostic factors for steroid-free remission in IIM.Objectives:We investigated the various clinical factors, including body measurements, that affect IIM treatment outcomes.Methods:Patients who were newly diagnosed with IIM between 2000 and 2018 were included. Steroid-free remission was defined as at least three months of normalisation of muscle enzymes and no detectable clinical disease activity. The factors associated with steroid-free remission were evaluated by a Cox regression analysis.Results:Of the 106 IIM patients, 35 displayed steroid-free remission during follow-up periods. In the multivariable Cox regression analyses, immunosuppressants’ early use within one month after diagnosis [hazard ratio (HR) 6.21, 95% confidence interval (CI) 2.61–14.74, p < 0.001] and sex-specific height quartiles (second and third quartiles versus first quartile, HR 3.65, 95% CI 1.40–9.51, p = 0.008 and HR 2.88, 95% CI 1.13–7.32, p = 0.027, respectively) were positively associated with steroid-free remission. Polymyositis versus dermatomyositis (HR 0.21, 95% CI 0.09–0.53, p = 0.001), presence of dysphagia (HR 0.15, CI 0.05–0.50, p = 0.002) and highest versus lowest quartile of waist circumference (WC) (HR 0.24, 95% CI 0.07–0.85, p = 0.027) were negatively associated with steroid-free remission.Conclusion:The early initiation of immunosuppressant therapy, type of myositis and presence of dysphagia are strong predictors of steroid-free remission in IIM; moreover, height and WC measurements at baseline may provide additional important prognostic value.Disclosure of Interests:None declared


2020 ◽  
Vol 12 ◽  
pp. 1759720X2093682
Author(s):  
Jung Sun Lee ◽  
Jung Eun Lee ◽  
Seokchan Hong ◽  
Chang-Keun Lee ◽  
Bin Yoo ◽  
...  

Background: Several studies have been conducted on factors associated with mortality in idiopathic inflammatory myopathies (IIMs), but few studies have assessed prognostic factors for steroid-free remission in IIM. We investigated the various clinical factors, including body measurements, that affect IIM treatment outcomes. Methods: Patients who were newly diagnosed with IIM between 2000 and 2018 were included. Steroid-free remission was defined as at least 3 months of normalisation of muscle enzymes and no detectable clinical disease activity. The factors associated with steroid-free remission were evaluated by a Cox regression analysis. Results: Of the 106 IIM patients, 35 displayed steroid-free remission during follow-up periods. In the multivariable Cox regression analyses, immunosuppressants’ early use within 1 month after diagnosis [hazard ratio (HR) 6.21, 95% confidence interval (CI) 2.61–14.74, p < 0.001] and sex-specific height quartiles (second and third quartiles versus first quartile, HR 3.65, 95% CI 1.40–9.51, p = 0.008 and HR 2.88, 95% CI 1.13–7.32, p = 0.027, respectively) were positively associated with steroid-free remission. Polymyositis versus dermatomyositis (HR 0.21, 95% CI 0.09–0.53, p = 0.001), presence of dysphagia (HR 0.15, CI 0.05–0.50, p = 0.002) and highest versus lowest quartile of waist circumference (WC; HR 0.24, 95% CI 0.07–0.85, p = 0.027) were negatively associated with steroid-free remission. Conclusion: The early initiation of immunosuppressant therapy, type of myositis and presence of dysphagia are strong predictors of steroid-free remission in IIM; moreover, height and WC measurements at baseline may provide additional important prognostic value.


2021 ◽  
Vol 20 ◽  
pp. 153303382110279
Author(s):  
Qinping Guo ◽  
Yinquan Wang ◽  
Jie An ◽  
Siben Wang ◽  
Xiushan Dong ◽  
...  

Background: The aim of our study was to develop a nomogram model to predict overall survival (OS) and cancer-specific survival (CSS) in patients with gastric signet ring cell carcinoma (GSRC). Methods: GSRC patients from 2004 to 2015 were collected from the Surveillance, Epidemiology, and End Results (SEER) database and randomly assigned to the training and validation sets. Multivariate Cox regression analyses screened for OS and CSS independent risk factors and nomograms were constructed. Results: A total of 7,149 eligible GSRC patients were identified, including 4,766 in the training set and 2,383 in the validation set. Multivariate Cox regression analysis showed that gender, marital status, race, AJCC stage, TNM stage, surgery and chemotherapy were independent risk factors for both OS and CSS. Based on the results of the multivariate Cox regression analysis, prognostic nomograms were constructed for OS and CSS. In the training set, the C-index was 0.754 (95% CI = 0.746-0.762) for the OS nomogram and 0.762 (95% CI: 0.753-0.771) for the CSS nomogram. In the internal validation, the C-index for the OS nomogram was 0.758 (95% CI: 0.746-0.770), while the C-index for the CSS nomogram was 0.762 (95% CI: 0.749-0.775). Compared with TNM stage and SEER stage, the nomogram had better predictive ability. In addition, the calibration curves also showed good consistency between the predicted and actual 3-year and 5-year OS and CSS. Conclusion: The nomogram can effectively predict OS and CSS in patients with GSRC, which may help clinicians to personalize prognostic assessments and clinical decisions.


2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Marrco Vitolo ◽  
Vincenzo Livio Malavasi ◽  
Marco Proietti ◽  
Igor Diemberger ◽  
Laurent Fauchier ◽  
...  

Abstract Aims Cardiac troponins (cTn) have been reported to be predictors for adverse outcomes in atrial fibrillation (AF), patients, but their actual use is still unclear. To assess the factors associated with cTn testing in routine clinical practice and to evaluate the association of elevated levels of cTn with adverse outcomes in a large contemporary cohort of European AF patients. Methods and results Patients enrolled in the ESC-EHRA EORP-AF General Long-Term Registry were stratified into three groups according to cTn levels as (i) cTn not tested, (ii) cTn in range (≤99th percentile), and (iii) cTn elevated (&gt;99th percentile). The composite outcome of any thromboembolism/any acute coronary syndrome (ACS)/cardiovascular (CV) death, defined as major adverse cardiovascular events (MACE) and all-cause death were the main endpoints. 10 445 (94.1%) AF patients were included in this analysis [median age 71 years, interquartile range (IQR): 63–77; males 59.7%]. cTn were tested in 2834 (27.1%). Overall, cTn was elevated in 904 (8.7%) and in-range in 1930 (18.5%) patients. Patients in whom cTn was tested tended to be younger (P &lt; 0.001) and more frequently presenting with first detected AF and atypical AF-related symptoms (i.e. chest pain, dyspnoea, or syncope) (P &lt; 0.001). On multivariable logistic regression analysis, female sex, in-hospital enrollment, first-detected AF, CV risk factors, history of coronary artery disease (CAD), and atypical AF symptoms were independently associated with cTn testing. After a median follow-up of 730 days (IQR: 692–749), 957 (9.7%) composite endpoints occurred while all-cause death was 9.5%. Kaplan–Meier analysis showed a higher cumulative risk for both outcomes in patients with elevated cTn levels (Figure) (Log Rank tests, P &lt; 0.001). On adjusted Cox regression analysis, elevated levels of cTn were independently associated with a higher risk for MACE [hazard ratio (HR): 1.74, 95% confidence interval (CI): 1.40–2.16] and all-cause death (HR 1.45, 95% CI: 1.21–1.74). Elevated levels of cTn were independently associated with a higher occurrence of MACE, all-cause death, any ACS, CV death and hospital readmission even after the exclusion of patients with history of CAD, diagnosis of ACS at discharge, those who underwent coronary revascularization during the admission and/or who were treated with oral anticoagulants plus antiplatelet therapy. Conclusions Elevated cTn levels were independently associated with an increased risk of all-cause mortality and adverse CV events, even after exclusion of CAD patients. Clinical factors that might enhance the need to rule out CAD were associated with cTn testing.


Vascular ◽  
2012 ◽  
Vol 20 (4) ◽  
pp. 198-202 ◽  
Author(s):  
Bahare Fazeli ◽  
Hassan Ravari ◽  
Reza Assadi

The aim of this study was first to describe the natural history of Buerger's disease (BD) and then to discuss a clinical approach to this disease based on multivariate analysis. One hundred eight patients who corresponded with Shionoya's criteria were selected from 2000 to 2007 for this study. Major amputation was considered the ultimate adverse event. Survival analyses were performed by Kaplan–Meier curves. Independent variables including gender, duration of smoking, number of cigarettes smoked per day, minor amputation events and type of treatments, were determined by multivariate Cox regression analysis. The recorded data demonstrated that BD may present in four forms, including relapsing-remitting (75%), secondary progressive (4.6%), primary progressive (14.2%) and benign BD (6.2%). Most of the amputations occurred due to relapses within the six years after diagnosis of BD. In multivariate analysis, duration of smoking of more than 20 years had a significant relationship with further major amputation among patients with BD. Smoking cessation programs with experienced psychotherapists are strongly recommended for those areas in which Buerger's disease is common. Patients who have smoked for more than 20 years should be encouraged to quit smoking, but should also be recommended for more advanced treatment for limb salvage.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Georg Schmidt ◽  
Axel Bauer ◽  
Petra Barthel ◽  
Alexander Müller

Background: Deceleration Capacity (DC) derived from 24-hour Holter recordings is a strong predictor of mortality in patients after acute myocardial infarction (MI). This prospective cohort study investigates the predictive power of DC derived from short-term recordings. Methods: 938 survivors of acute MI (age ≤80 years, sinus rhythm) were included. Within one week after index infarction, 30-min high resolution ECGs (1600Hz, supine position and resting conditions) as well as 24h-Holter recordings were performed. Short-term DC (DCs) and long-term DC (DCl) were calculated according to the previously published technology. Primary endpoint was death from any cause at two years. Multivariate Cox-regression analyses were adjusted for LVEF ≤30% and clinical covariates. Results: During the 2-year follow-up, 36 patients (3.8%) died. Both, DCs and DCl were strong predictors of death with areas under the receiver-operator characteristics curves of 76.3% and 77.8%, respectively (p<0.0001). DCs was significantly correlated with DCl (r=0.7, p<0.0001). After adjustment for LVEF, presence of diabetes mellitus, advanced age and history of a previous MI, DCs (≤2.5ms) remained a highly significant predictor of death (Table ). Conclusion: DC derived from high-resolution short-term ECGs is a strong and significant predictor of death in post-infarction patients. Multivariate Cox regression analysis for Prediction of 2y-Mortality


2019 ◽  
Vol 9 (1) ◽  
pp. 46 ◽  
Author(s):  
Caspar Mewes ◽  
Carolin Böhnke ◽  
Tessa Alexander ◽  
Benedikt Büttner ◽  
José Hinz ◽  
...  

Septic shock is a frequent life-threatening condition and a leading cause of mortality in intensive care units (ICUs). Previous investigations have reported a potentially protective effect of obesity in septic shock patients. However, prior results have been inconsistent, focused on short-term in-hospital mortality and inadequately adjusted for confounders, and they have rarely applied the currently valid Sepsis-3 definition criteria for septic shock. This investigation examined the effect of obesity on 90-day mortality in patients with septic shock selected from a prospectively enrolled cohort of septic patients. A total of 352 patients who met the Sepsis-3 criteria for septic shock were enrolled in this study. Body-mass index (BMI) was used to divide the cohort into 24% obese (BMI ≥ 30 kg/m2) and 76% non-obese (BMI < 30 kg/m2) patients. Kaplan-Meier survival analysis revealed a significantly lower 90-day mortality (31% vs. 43%; p = 0.0436) in obese patients compared to non-obese patients. Additional analyses of baseline characteristics, disease severity, and microbiological findings outlined further statistically significant differences among the groups. Multivariate Cox regression analysis estimated a significant protective effect of obesity on 90-day mortality after adjustment for confounders. An understanding of the underlying physiologic mechanisms may improve therapeutic strategies and patient prognosis.


Author(s):  
Carlo Rinaldi ◽  
Elena Salvatore ◽  
Ilaria Giordano ◽  
Sara De Matteis ◽  
Tecla Tucci ◽  
...  

Background:The primary aim of the present study was to determine the survival rates and identify predictors of disease duration in a cohort of Huntington's disease (HD) patients from Southern Italy.Methods:All medical records of HD patients followed between 1977 and 2008 at the Department of Neurological Sciences of Federico II University in Naples were retrospectively reviewed and 135 patients were enrolled in the analysis. At the time of data collection, 41 patients were deceased (19 males and 22 females) with a mean ± SD age at death of 56.6 ± 14.9 years (range 18-83).Results:The median survival time was 20 years (95% CI: 18.3-21.7). Cox regression analysis showed that the number of CAG in the expanded allele (HR 1.09 for 1 point triplet increase, p=0.002) and age of onset (HR 1.05 for 1 point year increase, p=0.022) were independent and significant predictors of lower survival rates.Conclusions:We believe that these findings are important for a better understanding of the natural history of the disease and may be relevant in designing future therapeutic trials.


2020 ◽  
Vol 9 (9) ◽  
pp. 3009
Author(s):  
José Antonio Rubio ◽  
Sara Jiménez ◽  
José Luis Lázaro-Martínez

Background: This study reviews the mortality of patients with diabetic foot ulcers (DFU) from the first consultation with a Multidisciplinary Diabetic Foot Team (MDFT) and analyzes the main cause of death, as well as the relevant clinical factors associated with survival. Methods: Data of 338 consecutive patients referred to the MDFT center for a new DFU during the 2008–2014 period were analyzed. Follow-up: until death or until 30 April 2020, for up to 12.2 years. Results: Clinical characteristics: median age was 71 years, 92.9% had type 2 diabetes, and about 50% had micro-macrovascular complications. Ulcer characteristics: Wagner grade 1–2 (82.3%), ischemic (49.2%), and infected ulcers (56.2%). During follow-up, 201 patients died (59.5%), 110 (54.7%) due to cardiovascular disease. Kaplan—Meier curves estimated a reduction in survival of 60% with a 95% confidence interval (95% CI), (54.7–65.3) at 5 years. Cox regression analysis adjusted to a multivariate model showed the following associations with mortality, with hazard ratios (HRs) (95% CI): age, 1.07 (1.05–1.08); HbA1c value < 7% (53 mmol/mol), 1.43 (1.02–2.0); active smoking, 1.59 (1.02–2.47); ischemic heart or cerebrovascular disease, 1.55 (1.15–2.11); chronic kidney disease, 1.86 (1.37–2.53); and ulcer severity (SINBAD system) 1.12 (1.02–1.26). Conclusion: Patients with a history of DFU have high mortality. Two less known predictors of mortality were identified: HbA1c value < 7% (53 mmol/mol) and ulcer severity.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Ahmadvazir ◽  
J Pradhan ◽  
R S Khattar ◽  
R Senior

Abstract Background The long-term clinical impact of carotid plaque burden (CPB) in patients with new onset suspected stable angina beyond stress echocardiography (SE) with no history of coronary artery disease (CAD) is not known. Methods Consecutive patients referred for SE, underwent simultaneous carotid ultrasonography to assess CPB. Patients were prospectively followed up for major adverse events (MAE). Results Of the 592 patients, 573 (age 59±11, 45% male) had follow-up data. During a mean of 7±1.2 years, 85 patients had first MAE (all-cause mortality and acute myocardial infarction: 67 (hard events) and 18 unplanned revascularisation). On multivariate Cox regression analysis, pre-test probability of CAD, peak wall thickness scoring index and CPB predicted MAE (p<0.0001 for all); however, only CPB retained significance for both hard events and hard cardiac events (p=0.008 and 0.001, respectively). MAE and hard events were least in patients with normal SE and absent carotid plaque (annualised event rate: 1.1% and 1.01%respectively) with significant increase in normal SE with plaque disease (2.4% and 2.05%, p=0.004 and 0.01 respectively). Presence of plaque did not impact on these outcomes in abnormal SE. Conclusions In patients with suspected stable angina, carotid atherosclerosis and myocardial ischemia in combination provided synergistic MAE information long term but atherosclerosis predicted hard events particularly in patients with normal SE but not in ischemic patients. This implies routine use of simultaneous carotid ultrasound following a normal SE for optimum prognostication


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