scholarly journals Survival of Patients With Idiopathic Inflammatory Myopathies in Slovenia

2021 ◽  
Vol 8 ◽  
Author(s):  
Alojzija Hočevar ◽  
Andrej Viršček ◽  
Monika Krošel ◽  
Suzana Gradišnik ◽  
Žiga Rotar ◽  
...  

Background: Idiopathic inflammatory myopathies (IIMs) are rare systemic diseases associated with significant morbidity and mortality. The aim of our study was to estimate for the first time the survival of IIM patients in Slovenia.Methods: We included IIM patients diagnosed between January 2005 and December 2020 and followed at two secondary/tertiary rheumatology centers in the country. To study survival/mortality the censor date of April 14 2021 was set. Kaplan–Meier analysis and standardized mortality ratio (SMR) were plotted using data of age and sex matched Slovenian population as a reference. Cox proportional hazards regression analysis was used to study prognostic factors for IIM mortality.Results: During the 16-year observation period, we identified 217 new IIM patients. During follow up 65 (30.0%) patients died. In the first year following IIM diagnosis the SMR was nearly 7-fold higher compared to the matched general population [SMR 6.88 (95%CI 4.41–10.24)] and remained higher also during the following 4 years. However, when excluding IIM patients with cancer, the survival outcome was, except in the first year after IIM diagnosis [SMR 5.55 (95%CI 3.10–9.15)], comparable to matched general population. In addition to cancer [HR 3.71 (95% CI 2.18–6.04)], cardiac involvement [HR 2.18 (95% CI 1.07–4.45)], fever [HR 2.13 (95% CI 1.13–4.03)], and older age [HR 1.07 (95% CI 1.04–1.09)] were extracted as prognostic factors associated with death.Conclusion: The survival of patients with IIM patients was substantially worse compared to matched general population. Cancer was the leading cause of death in our cohort.

2019 ◽  
Vol 46 (11) ◽  
pp. 1509-1514 ◽  
Author(s):  
Valérie Leclair ◽  
John Svensson ◽  
Ingrid E. Lundberg ◽  
Marie Holmqvist

Objective.Evidence suggests an increased risk of cardiovascular (CV) diseases, including acute coronary syndrome (ACS), in idiopathic inflammatory myopathies (IIM). The aim of this study was to investigate the risk of ACS in an incident IIM cohort compared to the general Swedish population.Methods.A cohort of 655 individuals with incident IIM and 6813 general population comparators were identified from national registries. IIM subjects were diagnosed from 2002 to 2011. Followup started at IIM diagnosis and corresponding date in the general population. ACS, CV comorbidities, and CV risk factors were defined using International Classification of Diseases codes. Incidence rates including 95% CI were calculated. Cox proportional hazards models were used to compare the risk of ACS in patients with IIM and the general population. The competing risk of death was accounted for using competing risk regression models.Results.The incidence rate of ACS in IIM was higher than in the general population, particularly within the first year of diagnosis and in older individuals. The overall ACS incidence rate in IIM was 15.6 (95% CI 11.7–20.4) per 1000 person-years, with an HR of 2.4 (95% CI 1.8–3.2) compared with the general population. When accounting for the competing risk of death, the risk of ACS in IIM remained increased with a cumulative incidence of 7% at 5 years compared to 3.3% in the general population.Conclusion.IIM individuals are at higher risk of ACS, particularly within the first year after diagnosis.


Stroke ◽  
2019 ◽  
Vol 50 (2) ◽  
pp. 487-490 ◽  
Author(s):  
Nicole L. De La Mata ◽  
Philip Masson ◽  
Rustam Al-Shahi Salman ◽  
Patrick J. Kelly ◽  
Angela C. Webster

Background and Purpose— People with end-stage kidney disease (ESKD) are at greater risk of stroke. We aimed to compare stroke mortality between the ESKD population and the general population. Methods— We included all patients with incident ESKD in Australia, 1980 to 2013, and New Zealand, 1988 to 2012. The primary cause of death was ascertained using data linkage with national death registers. We produced standardized mortality ratios for stroke deaths, by age, sex, and calendar year. Results— We included 60 823 patients with ESKD, where 941 stroke deaths occurred during 381 874 person-years. Patients with ESKD had >3× the stroke deaths compared with the general population (standardized mortality ratio, 3.4; 95% CI, 3.2–3.6), markedly higher in younger people and women. The greatest excess was in intracerebral hemorrhages (standardized mortality ratio, 5.2; 95% CI, 4.5–5.9). Excess stroke deaths in patients with ESKD decreased over time, although were still double in 2013 (2013 standardized mortality ratio, 2.1; 95% CI, 1.5–2.9). Conclusions— People with ESKD experience much greater stroke mortality with the greatest difference for women and younger people. However, mortality has improved over time.


2019 ◽  
Vol 188 (7) ◽  
pp. 1371-1382 ◽  
Author(s):  
Henry T Zhang ◽  
Leah J McGrath ◽  
Alan R Ellis ◽  
Richard Wyss ◽  
Jennifer L Lund ◽  
...  

Abstract Nonexperimental studies of the effectiveness of seasonal influenza vaccine in older adults have found 40%–60% reductions in all-cause mortality associated with vaccination, potentially due to confounding by frailty. We restricted our cohort to initiators of medications in preventive drug classes (statins, antiglaucoma drugs, and β blockers) as an approach to reducing confounding by frailty by excluding frail older adults who would not initiate use of these drugs. Using a random 20% sample of US Medicare beneficiaries, we framed our study as a series of nonrandomized “trials” comparing vaccinated beneficiaries with unvaccinated beneficiaries who had an outpatient health-care visit during the 5 influenza seasons occurring in 2010–2015. We pooled data across trials and used standardized-mortality-ratio–weighted Cox proportional hazards models to estimate the association between influenza vaccination and all-cause mortality before influenza season, expecting a null association. Weighted hazard ratios among preventive drug initiators were generally closer to the null than those in the nonrestricted cohort. Restriction of the study population to statin initiators with an uncensored approach resulted in a weighted hazard ratio of 1.00 (95% confidence interval: 0.84, 1.19), and several other hazard ratios were above 0.95. Restricting the cohort to initiators of medications in preventive drug classes can reduce confounding by frailty in this setting, but further work is required to determine the most appropriate criteria to use.


Rheumatology ◽  
2021 ◽  
Author(s):  
Yuichi Yamasaki ◽  
Norimoto Kobayashi ◽  
Shinji Akioka ◽  
Kazuko Yamazaki ◽  
Shunichiro Takezaki ◽  
...  

Abstract Objectives This study aimed to investigate the clinical characteristics, treatment and prognosis of juvenile idiopathic inflammatory myopathies (JIIM) in Japan for each myositis-specific autoantibody (MSA) profile. Methods A multicentre, retrospective study was conducted using data of patients with JIIM at nine paediatric rheumatology centres in Japan. Patients with MSA profiles, determined by immunoprecipitation using stored serum from the active stage, were included. Results MSA were detected in 85 of 96 cases eligible for the analyses. Over 90% of the patients in this study had one of the following three MSA types: anti-melanoma differentiation-associated protein 5 (MDA5) (n = 31), anti-transcriptional intermediary factor 1 alpha and/or gamma subunits (TIF1γ) (n = 25) and anti-nuclear matrix protein 2 (NXP2) (n = 25) antibodies. Gottron papules and periungual capillary abnormalities were the most common signs of every MSA group in the initial phase. The presence of interstitial lung disease (ILD) was the highest risk factor for patients with anti-MDA5 antibodies. Most patients were administered multiple drug therapies: glucocorticoids and MTX were administered to patients with anti-TIF1γ or anti-NXP2 antibodies. Half of the patients with anti-MDA5 antibodies received more than three medications including i.v. CYC, especially patients with ILD. Patients with anti-MDA5 antibodies were more likely to achieve drug-free remission (29 vs 21%) and less likely to relapse (26 vs 44%) than others. Conclusion Anti-MDA5 antibodies are the most common MSA type in Japan, and patients with this antibody are characterized by ILD at onset, multiple medications including i.v. CYC, drug-free remission, and a lower frequency of relapse. New therapeutic strategies are required for other MSA types.


2021 ◽  
Vol 18 (1) ◽  
Author(s):  
Volkan Korten ◽  
◽  
Deniz Gökengin ◽  
Gülhan Eren ◽  
Taner Yıldırmak ◽  
...  

Abstract Background There is limited evidence on the modification or stopping of antiretroviral therapy (ART) regimens, including novel antiretroviral drugs. The aim of this study was to evaluate the discontinuation of first ART before and after the availability of better tolerated and less complex regimens by comparing the frequency, reasons and associations with patient characteristics. Methods A total of 3019 ART-naive patients registered in the HIV-TR cohort who started ART between Jan 2011 and Feb 2017 were studied. Only the first modification within the first year of treatment for each patient was included in the analyses. Reasons were classified as listed in the coded form in the web-based database. Cumulative incidences were analysed using competing risk function and factors associated with discontinuation of the ART regimen were examined using Cox proportional hazards models and Fine-Gray competing risk regression models. Results The initial ART regimen was discontinued in 351 out of 3019 eligible patients (11.6%) within the first year. The main reason for discontinuation was intolerance/toxicity (45.0%), followed by treatment simplification (9.7%), patient willingness (7.4%), poor compliance (7.1%), prevention of future toxicities (6.0%), virologic failure (5.4%), and provider preference (5.4%). Non-nucleoside reverse transcriptase inhibitor (NNRTI)-based (aHR = 4.4, [95% CI 3.0–6.4]; p < 0.0001) or protease inhibitor (PI)-based regimens (aHR = 4.3, [95% CI 3.1–6.0]; p < 0.0001) relative to integrase strand transfer inhibitor (InSTI)-based regimens were significantly associated with ART discontinuation. ART initiated at a later period (2015-Feb 2017) (aHR = 0.6, [95% CI 0.4–0.9]; p < 0.0001) was less likely to be discontinued. A lower rate of treatment discontinuation for intolerance/toxicity was observed with InSTI-based regimens (2.0%) than with NNRTI- (6.6%) and PI-based regimens (7.5%) (p < 0.001). The percentage of patients who achieved HIV RNA < 200 copies/mL within 12 months of ART initiation was 91% in the ART discontinued group vs. 94% in the continued group (p > 0.05). Conclusion ART discontinuation due to intolerance/toxicity and virologic failure decreased over time. InSTI-based regimens were less likely to be discontinued than PI- and NNRTI-based ART.


2020 ◽  
Vol 8 (Suppl 3) ◽  
pp. A7-A7
Author(s):  
Soraia Lobo-Martins ◽  
Diogo Martins-Branco ◽  
Patrícia Miguel Semedo ◽  
Cecília Melo Alvim ◽  
Ana Maria Monteiro ◽  
...  

BackgroundImmune checkpoint inhibitors (ICI) have changed the paradigm of advanced malignant melanoma (MM). Several prognostic factors, mostly linked to inflammation, have been under scope to better select patients for such therapies. We aimed to build and apply a prognostic score in this setting.MethodsBaseline characteristics and outcomes on 147 patients with advanced MM treated with an anti-PD1 (nivolumab or pembrolizumab) in monotherapy, between Jan-2016 and Oct-2019, in the 1st, 2nd or 3rd line setting were collected from two centres in Portugal. Data cut-off for follow-up was May-2020. Cox proportional hazards regression was used to identify independent prognostic factors for OS.ResultsWith a median FU of 28.93 months (95% CI [22.52–33.54]), mOS for the whole cohort was 14.75 months (95% CI, [10.80–18.71]). Overall, 43 and 104 patients were treated with nivolumab and pembrolizumab, respectively. We identified four adverse prognostic factors that were independent predictors of bad prognosis: number of metastatic sites >2 (p<0.001), baseline PS-ECOG =1 (p<0.001), presence of baseline lymphopenia (over lower limit of normal) (p=0.002) or very high baseline LDH (>2x upper limit of normal) (p<0.001).Patients were separated into three risk categories according to the number of risk factors present: favourable prognosis (no risk factors; n=34), intermediate prognosis (one risk factor; n=65) and poor prognosis (two or more risk factors; n=48). mOS was 43.41 (95% CI [32.13–54.69], 14.39 (95% CI [6.78–22.01]) and 6.53 months (95% CI [3.61–9.44]), for favourable, intermediate, and poor prognosis group, respectively (p<0.001; figure 1). AUC of ROC curve for OS was 0.737 (95% CI [0.654–0.819], p<0.001).Abstract 7 Figure 1Time to death - Kaplan-Meier survival plotConclusionsUsing easily accessible parameters from our daily practice, we propose the MELImmune prognostic score for advanced MM patients treated with anti-PD1 in monotherapy that could be incorporated to the daily clinical practice and clinical trials. We further aim to validate this score in an independent larger sample.Ethics ApprovalThe study was approved by both institutions’ Ethics Committee.


2020 ◽  
Vol 4 (Supplement_1) ◽  
pp. 161-161
Author(s):  
Jane Banaszak-Holl ◽  
Xiaoping Lin ◽  
Jing Xie ◽  
Stephanie Ward ◽  
Henry Brodaty ◽  
...  

Abstract Research Aims: This study seeks to understand whether those with dementia experience higher risk of death, using data from the ASPREE (ASPirin in Reducing Events in the Elderly) clinical trial study. Methods: ASPREE was a primary intervention trial of low-dose aspirin among healthy older people. The Australian cohort included 16,703 dementia-free participants aged 70 years and over at enrolment. Participants were triggered for dementia adjudication if cognitive test results were poorer than expected, self-reporting dementia diagnosis or memory problems, or dementia medications were detected. Incidental dementia was adjudicated by an international adjudication committee using the Diagnostic and Statistical Manual for Mental Disorders (DSM-IV) criteria and results of a neuropsychological battery and functional measures with medical record substantiation. Statistical analyses used a cox proportional hazards model. Results: As previously reported, 1052 participants (5.5%) died during a median of 4.7 years of follow-up and 964 participants had a dementia trigger, of whom, 575 (60%) were adjucated as having dementia. Preliminary analyses has shown that the mortality rate was higher among participants with a dementia trigger, regardless of dementia adjudication outcome, than those without (15% vs 5%, Χ2 = 205, p &lt;.001). Conclusion: This study will provide important analyses of differences in the hazard ratio for mortality and causes of death among people with and without cognitive impairment and has important implications on service planning.


2021 ◽  
Vol 44 (4) ◽  
pp. 145-152
Author(s):  
Hualei Guo ◽  
Hao Chen ◽  
Wenhui Wang ◽  
Lingna Chen

Objective: The aim of this study was to investigate the clinicopathological prognostic factors of malignant ovarian germ cell tumors (MOGCT) and evaluate the survival trends of MOGCT by histotype. Methods: We extracted data on 1,963 MOGCT cases diagnosed between 2000 and 2014 from the Surveillance, Epidemiology, and End Results (SEER) database and the histological classification of MOGCT, including 5 categories: dysgerminoma, embryonal carcinoma (EC), yolk sac tumor, malignant teratoma, and mixed germ cell tumor. We examined overall and disease-specific survival of the 5 histological types. Kaplan-Meier and Cox proportional hazards regression models were used to estimate survival curves and prognostic factors. We also estimated survival curves of MOGCT according to different treatments. Results: There was a significant difference in prognosis among different histological classifications. Age, histotype, grade, SEER stage, and surgery were independent prognostic factors for survival of patients with MOGCT. For all histotypes, 1-, 3-, and 5-year survival rate estimates were >85%, except for EC, which had the worst outcomes at 1 year (55.6%), 3 years (44.4%), and 5 years (33.3%). In the distant SEER stage, both chemotherapy and surgery were associated with improved survival outcomes compared with surgery- and chemotherapy-only groups. Conclusions: Dysgerminoma patients had the most favorable outcomes, whereas EC patients had the worst survival. A young age, low grade, and surgery were all significant predictors for improved survival. In contrast, a distant SEER stage was a risk factor for poor survival. Chemotherapy combined with surgery contributed to longer survival times of patients with MOGCT in the distant SEER stage.


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


2015 ◽  
Vol 33 (11) ◽  
pp. 1243-1251 ◽  
Author(s):  
Sean O'Farrell ◽  
Hans Garmo ◽  
Lars Holmberg ◽  
Jan Adolfsson ◽  
Pär Stattin ◽  
...  

Purpose Findings on the association between risk of cardiovascular disease (CVD) and the duration and type of androgen-deprivation therapy (ADT) in men with prostate cancer (PCa) are inconsistent. Methods By using data on filled drug prescriptions in Swedish national health care registers, we investigated the risk of CVD in a cohort of 41,362 men with PCa on ADT compared with an age-matched, PCa-free comparison cohort (n = 187,785) by use of multivariable Cox proportional hazards regression models. Results From 2006 to 2012, 10,656 men were on antiandrogens (AA), 26,959 were on gonadotropin-releasing hormone (GnRH) agonists, and 3,747 underwent surgical orchiectomy. CVD risk was increased in men on GnRH agonists compared with the comparison cohort (hazard ratio [HR] of incident CVD, 1.21; 95% CI, 1.18 to 1.25; and orchiectomy: HR, 1.16; 95% CI, 1.08 to 1.25). Men with PCa on AA were at decreased risk (HR of incident CVD, 0.87; 95% CI, 0.82 to 0.91). CVD risk was highest during the first 6 months of ADT in men who experienced two or more cardiovascular events before therapy, with an HR of CVD during the first 6 months of GnRH agonist therapy of 1.91 (95% CI, 1.66 to 2.20), an HR of CVD with AA of 1.60 (95% CI, 1.24 to 2.06), and an HR of CVD with orchiectomy of 1.79 (95% CI, 1.16 to 2.76) versus the comparison cohort. Conclusion Our results support that there should be a solid indication for ADT in men with PCa so that benefit outweighs potential harm; this is of particular importance among men with a recent history of CVD.


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