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Background: This study aimed to describe the characteristics and outcomes of children and adolescents with autoimmune inflammatory rheumatic diseases (AIIRD) who were admitted to the pediatric intensive care unit (PICU). The accuracy of the Pediatric Risk of Mortality (PRISM) III and Pediatric Index of Mortality (PIM) 3 scores to predict the mortality were investigated., Methods: This was a retrospective cohort study. Children and adolescents with AIIRD aged ≤ 18 years who were admitted to the PICU at the largest university-based referral center in Thailand during July 2011 to June 2021 were included., Results: There were 122 PICU admissions from 74 patients; mean age of 12.0 ± 4.3 years, 74.3% female. Majority of AIIRD were systemic lupus erythematosus (SLE) (83.8%), followed by systemic juvenile idiopathic arthritis (5.4%), juvenile dermatomyositis (JDM) (2.7%) and microscopic polyangiitis (2.7%). The main cause of admission was combined infection and disease flare (29.5%). Pneumonia was the main site of infection. Acinetobacter baumanii was the most common causative agent. Macrophage activation syndrome occurred in 8 (6.5%) admissions. The mortality rate of PICU admissions was 14.8% from 18 deaths; 17 with SLE and 1 with JDM. Mechanical ventilation (aOR 24.07, 95%CI:1.33-434.91, P= 0.031), pneumothorax (aOR 24.08, 95%CI:1.76-328.86, P = 0.017 and thrombocytopenia (aOR 8.34, 95%CI:1.31-53.73, P = 0.025) were associated with mortality. The risk of mortality rate as predicted by the PRISM III score increased with a score ≥ 9. For the PIM 3 score, the risk of mortality increased if the score ≥ 3. The area under the ROC curve for the PRISM III and PIM 3 scores was 0.741 (95%CI: 0.633-0.849), P = 0.001 and 0.804 (95%CI: 0.685-0.924), P < 0.001, respectively. The model calibration using the Hosmer-Lemeshow goodness of fit test demonstrated a chi-square of 4.335, P = 0.826 for PRISM III and 7.987, P = 0.435 for PIM 3., Conclusion: SLE was the main AIIRD that required admission to the PICU. Mechanical ventilation, pneumothorax and thrombocytopenia were associated with mortality in pediatric patients with AIIRD. The PRISM III and PIM 3 scores demonstrated good calibration, while the PIM 3 score provided better discrimination ability in the prediction of mortality for pediatric AIIRD., Competing Interests: Declarations. Ethics approval and consent to participate: The protocol for this study received ethical approval from the Siriraj Institutional Review Board (SIRB) (COA no. Si 416/2021). Consent for publication: Not applicable. Competing interests: All authors declare no personal or professional conflicts of interest relating to any aspect of this study., (© 2025. The Author(s).)

Introduction: We aimed to evaluate the characteristics of the patients with a rheumatologic disease who underwent TPE., Method: A single-center, retrospective study was conducted between January 2016 and June 2023., Results: Twenty patients with a median age of 51 years received a median of 6 TPE sessions. Concurrently, immunosuppressive therapy was administered to 18 (90%) of them. During the follow-up period, 9 patients (45%) died. Creatinine (p = 0.001), C-reactive protein (p = 0.001), sedimentation rate (p = 0.002), leukocyte (p = 0.003), thrombocyte (p = 0.003), and neutrophil (p = 0.003) counts was decreased after TPE. Similarly, in the ROC analysis of post TPE laboratory parameters, urea, creatinine, CRP, hemoglobin, platelets, and lymphocytes predicted mortality with areas under the curve values ranging from 0.747 to 0.869. In the Cox regression analysis for mortality, creatinine was predictive for mortality (p = 0.030), HR 1.59 (95% CI: 1.05-2.41)., Conclusion: In rheumatologic conditions, TPE is beneficial to fill the gap until the effects of immunosuppressants become apparent., (© 2024 International Society for Apheresis and Japanese Society for Apheresis.)

Background & Objectives : To explore the potential association between positive ANA serology and all-cause mortality in a large cohort of patients, including those with and without rheumatological conditions and other immune-related diseases. Material and Methods : A retrospective cohort study analyzed all-cause mortality among 205,862 patients from Clalit Health Services (CHS), Israel's largest health maintenance organization (HMO). We compared patients aged 18 and older with positive ANA serology (n = 102,931) to an equal number of ANA-negative controls (n = 102,931). Multivariable Cox regression models were used to assess hazard ratios (HR) for mortality, adjusting for demographic and clinical factors. Results : ANA positivity was strongly associated with increased mortality (adjusted HR [aHR] 4.62; 95% CI 4.5-4.7, p < 0.001). Significant predictors of mortality included male gender (39.2% vs. 24.4%, p < 0.001), older age at testing (72.4 ± 13.0 vs. 50.1 ± 17.3 years, p < 0.001), and Jewish ethnicity (89.6% vs. 83.2%, p < 0.001). Certain ANA patterns, such as mitochondrial (and dense fine speckled (DFS-AC2)), were highly predictive of mortality, with aHRs of 36.14 (95% CI 29.78-43.85) and 29.77 (95% CI 26.58-33.34), respectively. ANA-positive patients with comorbid rheumatological immune-related disorders (RIRDs) demonstrated a higher survival rate compared to those without such a condition (aHR 0.9, 95% CI 0.86-0.95, p < 0.001). This finding remained significant after adjusting for several parameters, including age. Conclusions : ANA positivity is associated with increased all-cause mortality, particularly in individuals without rheumatologic disorders, after adjusting for confounders such as age. This may indicate occult malignancies, cardiovascular pathology, or chronic inflammatory states, necessitating more vigilant surveillance.

Objective: The objective of this study was to investigate the clinical manifestations and outcome of COVID-19 in patients with inflammatory rheumatic and musculoskeletal disease (iRMD) as compared with the general population., Methods: This is a case-control study of patients selected from the South Tyrol public health service, Italy, with and without iRMD, affected by COVID-19. We included patients ≥18 years and with a positive SARS-CoV-2 PCR test between 1 October 2020 and 1 March 2021. Cases were identified by linking the diagnosis of a rheumatic disease with PCR test positivity; these were then matched in a 1:1.8 (planned 1:2) ratio for age, sex, and date of COVID-19 diagnosis with people from the general population. The outcomes of primary interest were hospitalization, and severe course (intensive care unit, mechanical ventilation/extracorporeal membrane oxygenation, death)., Results: The study population consisted of 561 COVID-19 patients, of which 201 (mean age 60.4 years; 65.2% female) were patients with iRMD and 360 were controls from the general population (59.8 years; 64.7% female). The majority of iRMD patients (88.6%) were receiving an immunosuppressive drug at the time of COVID-19 diagnosis, and 36.3% were receiving glucocorticoids. COVID-19 related hospitalization (12.4% vs 10.6%, P = 0.49), severe course (5.0% vs 5.3%, P = 1.00), and mortality (3.5% vs 4.4%, P = 0.66) were similar between the groups. Among the hospitalized patients, mechanical ventilation was more common in iRMD patients than in controls [n = 5 (20.0%) vs n = 1 (2.6%), P = 0.035]., Conclusion: Our study indicated similar rates for admission, severe course, and mortality between patients with iRMD and controls affected by COVID-19. Among the hospitalized patients, mechanical ventilation was more frequently required in the iRMD group., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: Autoimmune or inflammatory rheumatic diseases (AIRDs) increase the risk for poor COVID-19 outcomes. Although rurality is associated with higher post-COVID-19 mortality in the general population, whether rurality elevates this risk among people with AIRD is unknown. We assessed associations between rurality and post-COVID-19 all-cause mortality, up to two years post infection, among people with AIRD using a large nationally sampled US cohort., Methods: This retrospective study used the National COVID Cohort Collaborative, a medical records repository containing COVID-19 patient data. We included adults with two or more AIRD diagnostic codes and a COVID-19 diagnosis documented between April 2020 and March 2023. Rural residency was categorized using patient residential zip codes. We adjusted for AIRD medications and glucocorticoid prescription, age, sex, race and ethnicity, tobacco or substance use, comorbid burden, and SARS-CoV-2 variant-dominant periods. Multivariable Cox proportional hazards with inverse probability treatment weighting assessed associations between rurality and two-year all-cause mortality., Results: Among the 86,467 SARS-CoV-2-infected persons with AIRD, we observed a higher risk for two-year post-COVID-19 mortality in rural versus urban dwellers. Rural-residing persons with AIRD had higher two-year all-cause mortality risk (adjusted hazard ratio 1.24, 95% confidence interval 1.19-1.29). Glucocorticoid, immunosuppressive, and rituximab prescriptions were associated with a higher risk for two-year post-COVID-19 mortality, whereas risk with nonbiologic or biologic disease-modifying antirheumatic drugs was lower., Conclusion: Rural residence in people with AIRD was independently associated with higher two-year post-COVID-19 mortality in a large US cohort after adjusting for background risk factors. Policymakers and health care providers should consider these findings when designing interventions to improve outcomes in people with AIRD following SARS-CoV-2 infection, especially among high-risk rural residents., (Published 2024. This article is a U.S. Government work and is in the public domain in the USA.)

Rheumatic diseases present unique challenges in the elderly, with changes in the immune system contributing to varied clinical presentations. More individuals are now living with chronic diseases due to greater life expectancy, but there is a lack of real-world data about rheumatic diseases and comorbidities in older people. This study aimed to investigate disease types, comorbidities, treatments, and mortality in geriatric patients in comparison to non-geriatric patients at a rheumatology clinic. This retrospective observational cohort study reviewed the medical records of 2610 patients from January 2021 to January 2024 at 2 branches of a private hospital's rheumatology clinics. Demographic information and data on rheumatic diseases, noninflammatory conditions, treatments, comorbidities, and mortality were collected, and geriatric patients were compared to non-geriatric patients. Geriatric patients (n = 515) had a significantly higher prevalence of rheumatoid arthritis (50.6% vs 28.8%, P < .001), polymyalgia rheumatica (11.1% vs 0.2%, P < .001), and crystal arthritis (19.6% vs 8.8%, P < .001), with more frequent geriatric-onset cases. Osteoarthritis was also more prevalent in geriatric patients (51.2% vs 11.3%, P < .001), while fibromyalgia was more common in the non-geriatric group (15.9% vs 4.1%, P < .001). Geriatric patients experienced higher rates of comorbidities, including hypertension (72.4% vs 17.8%, P < .001), diabetes (33.6% vs 12.1%, P < .001), and osteoporosis (64.9% vs 35.4%, P < .001). These patients used more corticosteroids (74.5% vs 44%, P < .001), and conventional synthetic disease-modifying antirheumatic drugs (62.4% vs 49.4%, P < .001) but fewer biological disease-modifying antirheumatic drugs (9.2% vs 23.1%, P < .001). Mortality rates were significantly higher in geriatric patients (6% vs 0.3%), with cancer (P = .001), ischemic heart disease (P = .04), heart failure (P = .01), chronic kidney disease (P = .02), and interstitial lung disease (P = .01) being associated with increased mortality. Geriatric rheumatology should receive greater focus in future research to help address the anticipated increases in demand and to develop tailored management strategies for elderly patients with rheumatic diseases and comorbidities., Competing Interests: The author has no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: SARS-CoV-2 infection has become a major international issue, not only from a medical point of view, but also social, economic and political. Most of the available information comes from the United States, Europe, and China, where the population and the socioeconomic status are very different from Latin American countries. This study evaluates the effect of regional socioeconomic characteristics on mortality due SARS-CoV-2 infection in patients with immune-mediated rheumatic diseases (IMRD) from Argentina, Mexico and Brazil., Methods: Data from three national registries, SAR-COVID (Argentina), CMR-COVID (Mexico) and ReumaCoV-Brasil (Brazil), were combined. Adult IMRD patients with SARS-CoV-2 infection were recruited. National data for each province/state, including population density, number of physicians per inhabitant, income, unemployment, GINI index, Municipal Human Development Index (MHDI), stringency index, vaccination rate and most frequent viral strains per period were assessed as risk factors for mortality due to COVID-19., Results: A total of 4744 patients were included, 2534 (53.4%) from SAR-COVID, 1166 (24.6%) from CMRCOVID and 1044 (22.0%) from ReumaCoV-Brasil. Mortality due to COVID-19 was 5.4%. In the multivariable analysis, higher number of physicians per 1000 inhabitants and being infected during the vaccination period of each country were associated with lower mortality. After adjustment for socioeconomic factors, there was no association with country of residence and mortality., Conclusion: These findings corroborate the complex interplay between socioeconomic factors, rheumatic disease activity, and regional disparities as determinants of death due to COVID-19 in Argentina, Brazil and Mexico. Thus, this research provides valuable insights for guiding public health policies and clinical practice in the ongoing fight against the COVID-19 pandemic., (© 2024. The Author(s).)

Objective: To analyze the long-term survival of subcutaneous biosimilar tumor necrosis factor inhibitors compared to the originator molecules in patients with rheumatic diseases, as well as the factors associated with drug discontinuation., Methods: Retrospective analysis of BIOBADASER, the Spanish multicenter prospective registry of patients with rheumatic disease receiving biologic and targeted disease-modifying antirheumatic drugs. Patients who started etanercept (ETN) or adalimumab (ADA) from January 2016 to October 2023 were included. The survival probabilities of biosimilars and originators were compared using Kaplan-Meier estimating curves. To identify factors associated with differences in the retention rates, hazard ratios (HR) were estimated using Cox regression models for all and specific causes (inefficacy or adverse events [AEs]) of discontinuation., Results: A total of 4162 patients received 4723 treatment courses (2991 courses of ADA and 1732 courses of ETN), of which 722 (15.29%) were with originator molecules and 4001 (84.71%) were with biosimilars. The originators were more frequently discontinued than biosimilars (53.32% vs 33.37%, respectively). The main reason for discontinuation was inefficacy (60.35% of the treatments). The risk of overall discontinuation was lower for biosimilars (adjusted HR 0.84, 95% CI 0.75-0.95). Female sex, obesity, and second or later treatment lines increased the risk of discontinuation, whereas disease duration and the use of concomitant methotrexate were associated with a greater survival. When assessing cause-specific reasons of discontinuation, excluding nonmedical switching, the results from the crude and adjusted analyses showed no significant differences in the retention rate between biosimilars and originators., Conclusion: No significant differences were found between treatments in long-term survival due to inefficacy or AEs., (Copyright © 2024 by the Journal of Rheumatology.)

Background: Malnutrition is a well-known risk factor for mortality among older adults. Arthritis and rheumatism are characterized by chronic inflammation and are also related to malnutrition as diagnosed using the Global Leadership Initiative on Malnutrition (GLIM) criteria. This study was thus developed to examine the associations linking malnutrition and all-cause death among older adults in China, employing the GLIM criteria to assess malnutrition., Methods: Two waves of the China Health and Retirement Longitudinal Study from 2013 and 2018 were used to conduct this study. Moderate malnutrition was defined as low BMI (< 18.5 and < 20 for individuals < 70 and 70 + years of age, respectively), an unintended 10-20% decrease in weight, or low muscle mass based on the sex-specific lowest 20% of the height-adjusted muscle mass as < 5.039 kg/m 2 in women and < 6.866 kg/m 2 in men. Severe malnutrition was defined as a > 20% unintended decrease in weight only or the combination of both low muscle mass and an unintended reduction of over 10% in weight. Associations between malnutrition and the risk of all-cause death were assessed through Cox regression analyses., Results: Overall, this study enrolled 1766 subjects 60 + years of age, of whom 57.36% (1033/1766) were female. Malnutrition was estimated to affect 418 (23.67%) of these individuals at baseline, with 21.06% and 2.60% affected by moderate and severe malnutrition, respectively. Over the 5-year follow-up, 189 of these individuals died. Covariate-adjusted Cox regression analyses confirmed a significant association between severe malnutrition and the risk of death in this cohort (HR = 2.196, 95%CI 1.125-4.286, P = 0.021)., Conclusions: Severe malnutrition, identified through screening based on the GLIM criteria, was associated with an increased risk of all-cause death among older Chinese adults with arthritis or rheumatism., (© 2024. The Author(s).)

Abstract: Hydroxychloroquine (HCQ) and chloroquine (CQ) are foundational treatments for several systemic autoimmune rheumatic diseases, including systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). Concerns regarding the risk of cardiac arrhythmia and death have been raised, yet the burden of HCQ and CQ-related cardiac toxicities remains unclear. A systematic literature search was conducted in the MEDLINE and Embase databases for articles published between the earliest date and April 2023 reporting cardiac conduction abnormalities in patients with systemic autoimmune rheumatic diseases taking HCQ or CQ. Meta-analysis was performed to calculate the difference in mean corrected QT (QTc) interval and odds ratio of prolonged QTc interval in those taking HCQ or CQ versus not. Of 2673 unique records, 34 met the inclusion criteria, including 70,609 subjects. Thirty-three studies reported outcomes in HCQ and 9 in CQ. Five studies reported outcomes in RA, 11 in SLE, and 18 in populations with mixed rheumatic diseases. Eleven studies reported mean QTc and OR for prolonged QTc for meta-analysis, all reporting outcomes in HCQ. There was a significant increase in mean QTc (10.29 ms,  P  = 0.458) among HCQ users compared to non-HCQ users in patients with RA. There was no difference in mean QTc between HCQ and non-HCQ users in other systemic autoimmune rheumatic diseases. When rheumatic diseases were pooled, HCQ users were more likely to have prolonged QTc compared to non-HCQ users (odds ratio 1.57, 95% CI, 1.19, 2.08). The results of this study suggest that clinicians should be aware of potential adverse cardiac events of HCQ and consider QTc monitoring for patients on HCQ for the treatment of systemic autoimmune rheumatic diseases., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: Infectious conditions are a significant cause of mortality in autoimmune rheumatic diseases (ARD). Among patients hospitalized with an infection, we compared in-hospital and long-term (3-year) mortality between those with and without ARD., Methods: This retrospective analysis included members of the largest health maintenance organization in Israel, aged > 18 years at the first episode of infection, who required hospitalization during 2003-2019. We compared in-hospital mortality and the results of a 3-year landmark analysis of those who survived the index hospitalization between patients with ARD, according to disease subgroups, and patients without ARD. Additionally, we compared mortality outcomes among patients with ARD, according to subgroup diagnosis, matched in a 1:3 ratio by age, sex, and ethnicity to patients without ARD., Results: Included were 365,247 patients who were admitted for the first time with the diagnosis of a serious infection. Of these, we identified 9755 with rheumatoid arthritis (RA), 1351 with systemic lupus erythematosus, 2120 with spondyloarthritis (SpA), 584 with systemic sclerosis, and 3214 with vasculitis. In a matched multivariate analysis, the risk for in-hospital mortality was lower among patients with RA (odds ratio [OR] 0.89, 95% CI 0.81-0.97) and SpA (OR 0.77, 95% CI 0.63-0.94). In a similar analysis, the risk of 3-year mortality was lower among patients with RA (hazard ratio [HR] 0.82, 95% CI 0.78-0.86) and vasculitis (HR 0.86, 95% CI 0.80-0.93)., Conclusion: Among patients hospitalized for an infection, the risk of in-hospital and 3-year mortality was not increased among those with ARD compared to those without ARD., (Copyright © 2024 by the Journal of Rheumatology.)

Background/aim: Immunosuppressive and immunomodulatory treatments developed in recent years as a result of a better understanding of the pathophysiology of systemic rheumatic diseases (SRDs) improve the prognosis. Despite medical advances, individuals with SRDs at any stage may require intensive care and have a high mortality rate. The aim of this study was to investigate the demographic and clinical characteristics of patients with rheumatic diseases admitted to the intensive care unit (ICU), and the factors associated with the risk of mortality., Materials and Methods: This was a retrospective, cross-sectional study that included patients with rheumatic diseases in the medical ICU. Factors of ICU 28-day mortality were identified by multiple-variable logistic analysis., Results: A total of 127 patients with SRDs admitted to the medical ICU were enrolled. Systemic lupus erythematosus (SLE) (32.3%) was the most common diagnosis of SRDs in patients admitted to the ICU. The reasons for admission to the ICU were combined infection and primary SRD flare-up (35.4%), primary SRD flare-up (22%), SRD-unrelated reasons (22%), infection (17.3%), drug side effects (3.9%), and SRD-related complications (0.8%). The most common organ dysfunctions before (49.6%) and during (77.2%) admission to ICU were in the respiratory system. The 28-day mortality was 78 (61.4%). While the maximum procalcitonin, serum lactate, and blood urea nitrogen (BUN) levels were higher in the nonsurvivor group, the platelet and serum albumin levels were statistically significantly lower than those in the survivor group (p < 0.05). Acute respiratory failure (ARF), the presence of septic shock, the need for invasive mechanical ventilation (IMV), BUN level, and low platelet-lymphocyte ratio (PLR) were significant in the final multiple-variable model., Conclusion: Significant predictors of mortality in patients with rheumatic diseases may include ARF, septic shock, the need for IMV, and high BUN and low PLR levels., Competing Interests: Conflicts of interest: There are no conflicts of interest., (© TÜBİTAK.)

Objectives: To calculate the rates of COVID-19 infection and COVID-19-related death among people with rare autoimmune rheumatic diseases (RAIRD) during the first wave of the COVID-19 pandemic in England compared with the general population., Methods: We used Hospital Episode Statistics to identify all people alive on 1 March 2020 with ICD-10 codes for RAIRD from the whole population of England. We used linked national health records (demographic, death certificate, admissions and PCR testing data) to calculate rates of COVID-19 infection and death up to 31 July 2020. Our primary definition of COVID-19-related death was mention of COVID-19 on the death certificate. General population data from Public Health England and the Office for National Statistics were used for comparison. We also describe COVID-19-related hospital admissions and all-cause deaths., Results: We identified a cohort of 168 680 people with RAIRD, of whom 1874 (1.11%) had a positive COVID-19 PCR test. The age-standardized infection rate was 1.54 (95% CI: 1.50, 1.59) times higher than in the general population. A total of 713 (0.42%) people with RAIRD died with COVID-19 on their death certificate and the age-sex-standardized mortality rate for COVID-19-related death was 2.41 (2.30-2.53) times higher than in the general population. There was no evidence of an increase in deaths from other causes in the RAIRD population., Conclusions: During the first wave of COVID-19 in England, people with RAIRD had a 54% increased risk of COVID-19 infection and more than twice the risk of COVID-19-related death compared with the general population. These increases were seen despite shielding policies., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Abstract: Patients with systemic rheumatic disease (SRD) share the risks of multi-organ flare-up, cardiovascular diseases, and immunosuppression. Such situations can lead to an acute critical illness. The present study describes the clinical features of SRD patients admitted to the intensive care unit (ICU) and their short- and long- term mortality.We performed a multicentre retrospective study in 10 French ICU in Lyon, France. Inclusion criteria were SRD diagnosis and admission for an acute organ failure. The primary endpoint was ICU mortality.A total of 271 patients were included. SRD included systemic lupus erythematosus (23.2% of included patients), vasculitis (10.7%), systemic sclerosis (10.7%), idiopathic inflammatory myopathy (6.3%), and other connective tissue disorders (rheumatoid arthritis, Sjögren and Sharp syndromes; 50.9%). Initial organ failure(s) were shock (43.5% of included patients), acute kidney injury (30.5%), and acute respiratory failure (23.2%). The cause(s) of ICU admission included sepsis (61.6%), cardiovascular events (33.9%), SRD-flare up (32.8%), and decompensations related to comorbidities (28%). The ICU mortality reached 14.3%. The factors associated with ICU mortality were chronic cardiac failure, invasive ventilation and admission in ICU for another reason than sepsis or SRD flare-up. The median follow-up after ICU discharge was 33.6 months. During follow-up, 109 patients died. The factors associated with long-term mortality included age, Charlson comorbidity index, and ICU admission for sepsis or SRD flare-up.The ICU mortality of patients with SRD was low. Sepsis was the first cause of admission. Cardiovascular events and comorbidities negatively impacted ICU mortality. Admission for sepsis or SRD flare-up exerted a negative effect on the long-term outcome., Competing Interests: The authors have no funding and conflicts of interests to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Patients with inflammatory bowel disease, psoriasis or other rheumatic diseases treated with corticosteroids, immunomodulators and biologics might face additional risk during COVID-19 epidemic due to their immunocompromised status. However, there was still no unanimous opinion on the use of these therapy during COVID-19 epidemic. Current studies suggested that systemic corticosteroids might increase the risk of hospitalization, as well as risks of ventilation, ICU, and death among patients with immune-mediated inflammatory diseases. Anti-TNF agent was associated with lower rate of hospitalization, as well as lower risks of ventilation, ICU, and death. No significant changes in rates of hospitalization, ventilation, ICU and mortality were observed in patients treated with immunomodulators or biologics apart from anti-TNF agents. The underlying mechanism of these results might be related to pathway of antiviral immune response and cytokine storm induced by SARS-COV-2 infection. Decision on the use of corticosteroids, immunomodulators and biologics should be made after weighing the benefits and potential risks based on individual patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Zhang, Bai, Cao, Ji, Wang, Yang and Yang.)

Objectives: To determine factors associated with COVID-19-related death in people with rheumatic diseases., Methods: Physician-reported registry of adults with rheumatic disease and confirmed or presumptive COVID-19 (from 24 March to 1 July 2020). The primary outcome was COVID-19-related death. Age, sex, smoking status, comorbidities, rheumatic disease diagnosis, disease activity and medications were included as covariates in multivariable logistic regression models. Analyses were further stratified according to rheumatic disease category., Results: Of 3729 patients (mean age 57 years, 68% female), 390 (10.5%) died. Independent factors associated with COVID-19-related death were age (66-75 years: OR 3.00, 95% CI 2.13 to 4.22; >75 years: 6.18, 4.47 to 8.53; both vs ≤65 years), male sex (1.46, 1.11 to 1.91), hypertension combined with cardiovascular disease (1.89, 1.31 to 2.73), chronic lung disease (1.68, 1.26 to 2.25) and prednisolone-equivalent dosage >10 mg/day (1.69, 1.18 to 2.41; vs no glucocorticoid intake). Moderate/high disease activity (vs remission/low disease activity) was associated with higher odds of death (1.87, 1.27 to 2.77). Rituximab (4.04, 2.32 to 7.03), sulfasalazine (3.60, 1.66 to 7.78), immunosuppressants (azathioprine, cyclophosphamide, ciclosporin, mycophenolate or tacrolimus: 2.22, 1.43 to 3.46) and not receiving any disease-modifying anti-rheumatic drug (DMARD) (2.11, 1.48 to 3.01) were associated with higher odds of death, compared with methotrexate monotherapy. Other synthetic/biological DMARDs were not associated with COVID-19-related death., Conclusion: Among people with rheumatic disease, COVID-19-related death was associated with known general factors (older age, male sex and specific comorbidities) and disease-specific factors (disease activity and specific medications). The association with moderate/high disease activity highlights the importance of adequate disease control with DMARDs, preferably without increasing glucocorticoid dosages. Caution may be required with rituximab, sulfasalazine and some immunosuppressants., Competing Interests: Competing interests: AS reports personal fees from lectures for AbbVie, MSD, Roche, BMS and Pfizer, all outside the submitted work. MG reports grants from National Institutes of Health, NIAMS, outside the submitted work. JL reports a research grant from Pfizer, outside of the submitted work. EFM reports that LPCDR received support for specific activities: grants from Abbvie, Novartis, Janssen-Cilag, Lilly Portugal, Sanofi, Grünenthal S.A., MSD, Celgene, Medac, Pharmakern and GAfPA; grants and non-financial support from Pfizer; non-financial support from Grünenthal GmbH, outside the submitted work. CR has received consulting/speaker’s fees from Abbvie, Amgen, AstraZeneca, BMS, Biogen, Eli Lilly, Glenmark, GSK, MSD, Mylan and Pfizer, and grants from Biogen, Lilly and Nordic Pharma, all unrelated to this manuscript. MJS is supported by unrestricted grants from AbbVie, Biogen, Gilead, Lilly, MSD, Novartis and Pfizer. Her work is supported by grants from the National Institutes of Health and Agency for Healthcare Research and Quality. She leads the Data Analytic Center for the American College of Rheumatology, which is unrelated to this work. ES reports non-financial support from Canadian Arthritis Patient Alliance, outside the submitted work. JS is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (grant numbers K23 AR069688, R03 AR075886, L30 AR066953, P30 AR070253 and P30 AR072577), the Rheumatology Research Foundation (R Bridge Award), the Brigham Research Institute, and the R. Bruce and Joan M. Mickey Research Scholar Fund. He has received research support from Amgen and Bristol-Myers Squibb and performed consultancy for Bristol-Myers Squibb, Gilead, Inova Diagnostics, Janssen, Optum and Pfizer unrelated to this work. PS reports personal fees from American College of Rheumatology/Wiley Publishing, outside the submitted work. TT reports personal fees for lectures and expertises from Amgen, Arrow, Biogen, BMS, Chugai, Expanscience, Gilead, Grunenthal, LCA, Lilly, Medac, MSD, Nordic, Novartis, Pfizer, Sandoz, Sanofi, Theramex, Thuasne, TEVA and UCB, and reports financial support or fees for research activities from Amgen, Bone Therapeutics, Chugai, MSD, Novartis, Pfizer and UCB, all unrelated to this manuscript. ZSW reports grant support from Bristol-Myers Squibb and consulting fees from Viela Bio. JB-C has received consulting/speaker’s fees from Abbvie, MSD, BMS and Roche, and grants from Pfizer, all unrelated to this manuscript. He reports non-branded marketing campaigns for Novartis. PC has received consulting and lecturing fees from Abbvie, AstraZeneca, Bristol-Myers Squibb, Gilead, Glaxo Smith Kline, Innotech, Janssen, Merck Sharp Dohme, Roche, Servier and Vifor. LC has not received fees or personal grants from any laboratory, but her institute works by contract for laboratories among other institutions, such as Abbvie Spain, Eisai, Gebro Pharma, Merck Sharp & Dohme España, S.A., Novartis Farmaceutica, Pfizer, Roche Farma, Sanofi Aventis, Astellas Pharma, Actelion Pharmaceuticals España, Grünenthal GmbH and UCB Pharma. LG reports personal consultant fees from AbbVie, Amgen, BMS, Biogen, Celgene, Gilead, Janssen, Lilly, Novartis, Pfizer, Samsung Bioepis, Sanofi-Aventis and UCB, and grants from Amgen, Lilly, Janssen, Pfizer, Sandoz, Sanofi and Galapagos, all unrelated to this manuscript. RG reports non-financial support from Pfizer Australia, personal fees from Pfizer Australia, personal fees from Cornerstones, personal fees from Janssen New Zealand, non-financial support from Janssen Australia, personal fees from Novartis, outside the submitted work. EH reports personal consultant fees from Actelion, Sanofi-Genzyme and GSK, and grants from GSK, all unrelated to this manuscript. RH reports research grant from Pfizer and personal fees from AbbVie, Pfizer, Novartis, Amgen, Mylan, Gilead, Medac and Takeda, all outside the submitted work. JH reports grants from Rheumatology Research Foundation, grants from Childhood Arthritis and Rheumatology Research Alliance (CARRA), personal fees from Novartis, outside the submitted work. KLH reports she has received non-personal speaker’s fees from Abbvie and grant income from BMS, UCB and Pfizer, all unrelated to this manuscript. KLH is supported by the NIHR Manchester Biomedical Research Centre. PCR reports personal fees from Abbvie, Eli Lilly, Gilead, Janssen, Novartis, Pfizer, Roche and UCB, non-financial support from BMS, research funding from Janssen, Novartis, Pfizer and UCB, all outside the submitted work. JY reports consulting fees from AstraZeneca and Eli Lilly, and grants from Pfizer, outside the submitted work. PMM has received consulting/speaker’s fees from Abbvie, BMS, Celgene, Eli Lilly, Janssen, MSD, Novartis, Orphazyme, Pfizer, Roche and UCB, all unrelated to this manuscript, and is supported by the National Institute for Health Research (NIHR), University College London Hospitals (UCLH) and Biomedical Research Centre (BRC)., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: The course of novel coronavirus disease 2019 (COVID-19) has been of special concern in patients with inflammatory rheumatic diseases (IRDs) due to the immune dysregulation that may be associated with these diseases and the medications used for IRDs, that may affect innate immune responses., Objective: In this cohort study, we aimed to report the disease characteristics and variables associated with COVID-19 outcome among Turkish patients with IRDs., Methods: Between April and June, 2020, 167 adult IRD patients with COVID-19 were registered from 31 centers in 14 cities in Turkey. Disease outcome was classified in 4 categories; (i) outpatient management, (ii) hospitalization without oxygen requirement, (iii) hospitalization with oxygen requirement, and (iv) intensive care unit (ICU) admission or death. Multivariable ordinal logistic regression analysis was conducted to determine variables associated with a worse outcome., Results: 165 patients (mean age: 50 ± 15.6 years, 58.2% female) were included. Twenty-four patients (14.5%) recovered under outpatient management, 141 (85.5%) were hospitalized, 49 (30%) required inpatient oxygen support, 22 (13%) were treated in the ICU (17 received invasive mechanic ventilation) and 16 (10%) died. Glucocorticoid use (OR: 4.53, 95%CI 1.65-12.76), chronic kidney disease (OR: 12.8, 95%CI 2.25-103.5), pulmonary disease (OR: 2.66, 95%CI 1.08-6.61) and obesity (OR: 3.7, 95%CI 1.01-13.87) were associated with a worse outcome. Biologic disease-modifying antirheumatic drugs (DMARDs) do not seem to affect COVID-19 outcome while conventional synthetic DMARDs may have a protective effect (OR: 0.36, 95%CI 0.17-0.75). Estimates for the associations between IRD diagnoses and outcome were inconclusive., Conclusions: Among IRD patients with COVID-19, comorbidities and glucocorticoid use were associated with a worse outcome, while biologic DMARDs do not seem to be associated with a worse outcome., Competing Interests: KT has served as a speaker for Gilead. GH has received grant/research support from Celgene and has served as a speaker for AbbVie, Celgene, Novartis, and UCB Pharma. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Esatoglu, Tascilar, Babaoğlu, Bes, Yurttas, Akar, Pehlivan, Akleylek, Tecer, Seyahi, Yuce-Inel, Alpay-Kanitez, Bodakci, Tekgoz, Colak, Bolek, Koca, Kalyoncu, Icacan, Ugurlu, Oz, Hamuryudan, Hatemi and the Turkish Society for Rheumatology COVID-19 Registry Investigators.)

Objective: Racial/ethnic minorities experience more severe outcomes of coronavirus disease 2019 (COVID-19) in the general US population. This study was undertaken to examine the association between race/ethnicity and COVID-19 hospitalization, ventilation status, and mortality in people with rheumatic disease., Methods: US patients with rheumatic disease and COVID-19 were entered into the COVID-19 Global Rheumatology Alliance physician registry between March 24, 2020 and August 26, 2020 were included. Race/ethnicity was defined as White, African American, Latinx, Asian, or other/mixed race. Outcome measures included hospitalization, requirement for ventilatory support, and death. Multivariable regression models were used to estimate odds ratios (ORs) and 95% confidence intervals (95% CIs) adjusted for age, sex, smoking status, rheumatic disease diagnosis, comorbidities, medication use prior to infection, and rheumatic disease activity., Results: A total of 1,324 patients were included, of whom 36% were hospitalized and 6% died; 26% of hospitalized patients required mechanical ventilation. In multivariable models, African American patients (OR 2.74 [95% CI 1.90-3.95]), Latinx patients (OR 1.71 [95% CI 1.18-2.49]), and Asian patients (OR 2.69 [95% CI 1.16-6.24]) had higher odds of hospitalization compared to White patients. Latinx patients also had 3-fold increased odds of requiring ventilatory support (OR 3.25 [95% CI 1.75-6.05]). No differences in mortality based on race/ethnicity were found, though power to detect associations may have been limited., Conclusion: Similar to findings in the general US population, racial/ethnic minorities with rheumatic disease and COVID-19 had increased odds of hospitalization and ventilatory support. These results illustrate significant health disparities related to COVID-19 in people with rheumatic diseases. The rheumatology community should proactively address the needs of patients currently experiencing inequitable health outcomes during the pandemic., (© 2020, American College of Rheumatology.)

Background: Hydroxychloroquine (HCQ) has been promoted as a potential treatment of coronavirus disease 2019 (COVID-19), but there are safety concerns., Objective: The purpose of this study was to determine the effects of HCQ treatment on QT interval., Methods: We retrospectively studied the electrocardiograms of 819 patients treated with HCQ for rheumatologic diseases from 2000 to 2020. The primary outcome was corrected QT (QTc) interval, by Bazett formula, during HCQ therapy., Results: Mean patient age was 64.0 ± 10.9 years, and 734 patients (90%) were men. Median dosage of HCQ was 400 mg daily, and median (25th-75th percentile) duration of HCQ therapy was 1006 (471-2075) days. Mean on-treatment QTc was 430.9 ± 31.8 ms. In total, 55 patients (7%) had QTc 470-500 ms, and 12 (1.5%) had QTc >500 ms. Chronic kidney disease (CKD), history of atrial fibrillation (AF), and heart failure were independent risk factors for prolonged QTc. In a subset of 591 patients who also had a pretreatment electrocardiogram, mean QTc increased from 424.4 ± 29.7 ms to 432.0 ± 32.3 ms (P <.0001) during HCQ treatment. Of these patients, 23 (3.9%) had either prolongation of QTc >15% or on-treatment QTc >500 ms. Over median 5.97 (3.33-10.11) years of follow-up, 269 patients (33%) died. QTc >470 ms during HCQ treatment was associated with a greater mortality risk (hazard ratio 1.78; 95% confidence interval 1.16-2.71; P = .008) in univariable but not in multivariable analysis., Conclusion: HCQ is associated with QT prolongation in a significant fraction of patients. The risk of QT prolongation is higher among patients with CKD, AF, and heart failure, who may benefit from greater scrutiny., (Published by Elsevier Inc.)

Aim: To evaluate the clinical and laboratory characteristics, prognostic factors, and outcome of adult rheumatic disease-associated macrophage activation syndrome (MAS)., Method: A multicenter retrospective study was performed across 4 tertiary hospitals in China between January 1, 2017 to December 31, 2019., Results: There were 61 rheumatic disease patients with MAS enrolled into this retrospective clinical study. Fever and hyperferritinemia are the most frequent clinical feature and laboratory abnormality in MAS patients. Serum ferritin > 6000 ng/mL (odds ratio [OR] = 9.46, 95% CI = 2.53-47.13, P = .005) and hemophagocytosis in bone marrow smear (OR = 11.12, 95%, CI = 3.29-50.65, P = .001) were the 2 most prominent predictive factors indicating MAS occurrence. The 90-day all-cause mortality rate of all rheumatic disease patients with MAS was 22.9% (hazards ratio [HR] = 2.15, 95% CI = 0.81-6.78, P = .05). Platelets < 100 × 10 9 /L (HR = 3.23, 95% CI = 2.51-4.81, P = .01) and ferritin > 6000ng/mL (HR = 6.12, 95% CI = 2.93-16.27, P = .005) were independent predictors of poor outcome in rheumatic disease-associated MAS., Conclusion: Macrophage activation syndrome could be a fatal complication in rheumatic disease. Patients presenting with unexplained fever, serum ferritin > 6000 ng/mL, hepatosplenomegaly and cytopenia at baseline should raise the suspicion of MAS. The presence of serum ferritin > 6000 ng/mL, hepatosplenomegaly and low number of platelets was associated with poor outcome., (© 2020 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Objective: There is emerging evidence that COVID-19 disproportionately affects people from racial/ethnic minority and low socioeconomic status (SES) groups. Many physicians across the globe are changing practice patterns in response to the COVID-19 pandemic. We sought to examine the practice changes among rheumatologists and what they perceive the impact to be on their most vulnerable patients., Methods: We administered an online survey to a convenience sample of rheumatologists worldwide during the initial height of the pandemic (between 8 April and 4 May 2020) via social media and group emails. We surveyed rheumatologists about their opinions regarding patients from low SES and racial/ethnic minority groups in the context of the COVID-19 pandemic. Mainly, what their specific concerns were, including the challenges of medication access; and about specific social factors (health literacy, poverty, food insecurity, access to telehealth video) that may be complicating the management of rheumatologic conditions during this time., Results: 548 rheumatologists responded from 64 countries and shared concerns of food insecurity, low health literacy, poverty and factors that preclude social distancing such as working and dense housing conditions among their patients. Although 82% of rheumatologists had switched to telehealth video, 17% of respondents estimated that about a quarter of their patients did not have access to telehealth video, especially those from below the poverty line. The majority of respondents believed these vulnerable patients, from racial/ethnic minorities and from low SES groups, would do worse, in terms of morbidity and mortality, during the pandemic., Conclusion: In this sample of rheumatologists from 64 countries, there is a clear shift in practice to telehealth video consultations and widespread concern for socially and economically vulnerable patients with rheumatic disease., Competing Interests: Competing interests: SI receives grant funds from the National Institute of Arthritis and Musculoskeletal and Skin Diseases; SMG grants and personal fees from Novartis—consulting/research support, Pfizer—consulting/research support, BMC Musculoskeletal Disorders—editorial board and Horizon—research support; DJ-K owns stocks in the following companies: Cytodyn, Walgreens, AstraZeneca. All other authors have declared that no competing interests exist., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Critically ill patients with systemic rheumatic disease (SRD) have benefited from better provision of rheumatic and critical care in recent years. Recent comprehensive data regarding in-hospital mortality rates and, most importantly, long-term outcomes are scarce., Research Question: The aim of this study was to assess short and long-term outcome of patients with SRD who were admitted to the ICU., Study Design and Methods: All records of patients with SRD who were admitted to ICU between 2006 and 2016 were reviewed. In-hospital and one-year mortality rates were assessed, and predictive factors of death were identified., Results: A total of 525 patients with SRD were included. Causes of admission were most frequently shock (40.8%) and acute respiratory failure (31.8%). Main diagnoses were infection (39%) and SRD flare-up (35%). In-hospital and one-year mortality rates were 30.5% and 37.7%, respectively. Predictive factors that were associated with in-hospital and one-year mortalities were, respectively, age, prior corticosteroid therapy, simplified acute physiology score II ≥50, need for invasive mechanical ventilation, or need for renal replacement therapy. Knaus scale C or D and prior conventional disease modifying antirheumatic drug therapy was associated independently with death one-year after ICU admission., Interpretation: Critically ill patients with SRD had a fair outcome after an ICU stay. Increased age, prior corticosteroid therapy, and severity of critical illness were associated significantly with short- and long-term mortality rates. The one-year mortality rate was also associated with prior health status and conventional disease modifying antirheumatic drug therapy., (Copyright © 2020 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

Objective: To investigate differences in manifestations and outcomes of coronavirus disease 2019 (COVID-19) infection between those with and without rheumatic disease., Methods: We conducted a comparative cohort study of patients with rheumatic disease and COVID-19 (confirmed by severe acute respiratory syndrome coronavirus 2 PCR), compared in a 1:2 ratio with matched comparators on age, sex and date of COVID-19 diagnosis, between 1 March and 8 April 2020, at Partners HealthCare System in the greater Boston, Massachusetts area. We examined differences in demographics, clinical features and outcomes of COVID-19 infection. The main outcomes were hospitalisation, intensive care admission, mechanical ventilation and mortality., Results: We identified 52 rheumatic disease patients with COVID-19 (mean age, 63 years; 69% female) and matched these to 104 non-rheumatic disease comparators. The majority (39, 75%) of patients with rheumatic disease were on immunosuppressive medications. Patients with and without rheumatic disease had similar symptoms and laboratory findings. A similar proportion of patients with and without rheumatic disease were hospitalised (23 (44%) vs 42 (40%)), p=0.50) but those with rheumatic disease required intensive care admission and mechanical ventilation more often (11 (48%) vs 7 (18%), multivariable OR 3.11 (95% CI 1.07 to 9.05)). Mortality was similar between the two groups (3 (6%) vs 4 (4%), p=0.69)., Conclusions: Patients with rheumatic disease and COVID-19 infection were more likely to require mechanical ventilation but had similar clinical features and hospitalisation rates as those without rheumatic disease. These findings have important implications for patients with rheumatic disease but require further validation., Competing Interests: Competing interests: EMG reports editor position at New England Journal of Medicine and royalties from the textbook Rheumatology. HKC reports research support from AstraZeneca and consultancy fees from Takeda, Selecta, GlaxoSmithKline and Horizon. JAS reports research support from Amgen and Bristol-Myers Squibb and consultancy fees from Bristol-Myers Squibb, Gilead, Inova, Janssen and Optum., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

In the current Thematic Issue of Current Vascular Pharmacology (CVP), entitled "Systemic Autoimmune Rheumatic Diseases and Cardiology", presented in two parts, Part 1 and Part 2, review articles are included from specialists in cardiology, rheumatology, immunology and related fields. These reviews discuss the cardiovascular complications of the main systemic Autoimmune Rheumatic Diseases (ARDs). For example, the underlying pathogenetic mechanisms, the role of cardiovascular imaging and recommendations for prevention and management. These articles place inflammation as the key process, linking cardiovascular complications with ARDs. From all these reviews, the conclusion is the need for collaboration between the disciplines of Rheumatology and Cardiology to establish the emerging field of Cardio- Rheumatology. This will aid to fine-tune risk stratification and optimize preventive strategies and pharmacological therapies for patients with ARDs., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

The increased risk of cardiovascular disease (CVD) among patients with autoimmune rheumatic diseases such as rheumatoid arthritis, spondyloarthritis and systemic lupus erythematosus has been extensively documented. Sub-clinical atherosclerosis can be assessed using various non-invasive imaging techniques. However, the mechanisms underlying the higher risk of atherosclerotic CVD in patients with autoimmune rheumatic diseases are not fully known, although they seem to include chronic low-grade systemic inflammation leading to prolonged endothelial activation, accompanied by a pro-thrombotic/pro-coagulant and autoantibody state. Furthermore, sub-clinical atherosclerosis is also influenced by other traditional risk factors for CVD. Including the individual components of the metabolic syndrome (MetS: obesity, impaired glucose metabolism, dyslipidemia and high blood pressure), the degree of which is higher in these patients than in controls. The aim of this narrative review is to discuss the CV manifestations and risk factors involved in the increased risk of CVD among patients with autoimmune rheumatic diseases., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Objectives: To evaluate the effectiveness and safety of lower-dose sulfamethoxazole/trimethoprim therapy (SMX/TMP) for Pneumocystis jirovecii pneumonia (PCP) in patients with systemic rheumatic diseases., Methods: In this multicenter retrospective study, we compared effectiveness and safety of SMX/TMP for the treatment of PCP among patients divided into three groups according to the initial dosage of SMX/TMP: the low, ≤10 mg/kg/day; the intermediate, 10-15 mg/kg/day; and the high and conventional, 15-20 mg/kg/day for TMP dose., Results: Eighty-one patients, including 22, 30, and 29 patients in the low-, the intermediate- and the high-dose group could be analyzed and the 30-day survival rate were 100%, 93.3%, and 96.7%, respectively (P = 0.28). There were significant dose-dependent increasing trends of severe adverse drug reactions (ADRs) for SMX/TMP that were graded as ≥3 according to the Common Terminology Criteria for Adverse Events. When stratified by presence of severe hypoxemia defined by alveolar-arterial O2 gradient ≥45 mmHg, the 30-day survival and treatment modification rate were similar among the three groups, but frequency of severe ADRs were significantly decreased in the low-dose group. The low-dose group was independently and negatively associated with treatment modification within 14 days and severe ADRs., Conclusions: Lower dose SMX/TMP therapy with ≤10 mg/kg/day for TMP was as effective as higher dose therapy for the treatment of PCP and associated with lower rates of treatment modification and severe ADRs in patients with systemic rheumatic diseases., (Copyright © 2018 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.)

Background: Autoimmune inflammatory disease increases the risk of diffuse large B-cell lymphoma (DLBCL) and marginal zone lymphoma (MZL), but findings for other mature B-cell malignancies are equivocal. Furthermore, it has been suggested that the increase in DLBCL is due to the activated B-cell (ABC) subtype; but data on this, and the impact of inflammatory co-morbidities on survival, are sparse and contradictory., Methods: Data are from an established UK population-based cohort. Patients (n = 6834) diagnosed between 01/2009 and 08/2015 are included; DLBCL (n = 1771), myeloma (n = 1760), chronic lymphocytic leukaemia (CLL, n = 1580), MZL (n = 936), and follicular lymphoma (FL, n = 787). Information on rheumatological disorders and deaths was obtained by record-linkage to nationally compiled Hospital Episode Statistics, with age-and sex-matched individuals (n = 68,340) from the same catchment population (˜4 million people) providing the comparator., Results: Significantly increased risks for DLBCL (OR = 2.3, 95% CI 1.8-2.8) and MZL (OR = 2.0, 95% CI 1.5-2.7) were found for those with rheumatological disorders; the site distribution of those with/without rheumatological conditions differing for DLBCL (p = 0.007) and MZL (p = 0.002). No increases in risk were observed for the remaining mature B-cell malignancies, and no associations with survival were detected for DLBCL (age-adjusted HR = 1.2, 95% CI 0.9-1.6) or MZL (age-adjusted HR = 1.0, 95% CI 0.6-1.9). Furthermore, whilst our findings provide evidence for an association with rheumatological disease severity for DLBCL, they offer little support for the notion that the association is driven by an increase in the incidence of the ABC subtype., Conclusion: Our findings support the hypothesis that the chronic activation and proliferation of specific B-cell populations which characterize autoimmune disease increase the potential for the lymphomagenic events that lead to DLBCL and MZL in both males and females; but have no impact on the development of CLL, FL or MM, or on survival., (Copyright © 2019 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Objective: To determine mortality and predictive factors for lower intestinal perforation (LIP) among patients with autoimmune rheumatic diseases., Methods: This retrospective, single-center, observational study analyzed mortality rates in 31 autoimmune rheumatic disease patients with LIP who were admitted to our hospital from January 2002 to June 2017. The primary outcome was the mortality rate during hospitalization., Results: The median age at the time of LIP was 61 years, and the survival rate at discharge was 64.5%. Eleven patients died of sepsis during hospitalization. Cox univariable analysis for mortality during hospitalization showed that absence of abdominal pain (hazard ratio (HR) 5.61, 95% confidence interval (CI) 1.38-22.9), higher age (HR 1.06, 95% CI 1.01-1.11), chronic kidney disease (HR 6.89, 95% CI 1.85-25.7), systemic vasculitis (HR 3.95, 95% CI 1.14-13.6), higher blood urea nitrogen (HR 1.02, 95% CI 1.01-1.04), higher serum creatinine (HR 1.41, 95% CI 1.06-1.87), and LIP due to malignancy (HR 14.3, 95% CI 1.95-105.1) significantly increased mortality., Conclusion: Abdominal pain was absent in 16% of LIP patients with autoimmune rheumatic diseases, and this absence was a poor prognostic factor in this cohort. Moreover, higher age, chronic kidney disease, systemic vasculitis, and LIP due to malignancy were associated with significantly increased mortality. Physicians should be aware of LIP in autoimmune disease patients with higher age, chronic kidney diseases, or systemic vasculitis even if patients reveal mild abdominal symptoms.

Aim: The clinical importance of the anti-Ro antibody has not been completely understood. This study investigated identification of the association between mortality and clinical features in patients with autoimmune rheumatic diseases and detectable anti-Ro antibody titers., Methods: We retrospectively analyzed a total of 336 patients with autoimmune rheumatic diseases and positive anti-Ro antibody titers from January 2012 to January 2015. Clinical manifestations and other autoantibodies detected during the follow-up period were identified. Cumulative survival rates were assessed by Kaplan-Meier analysis. Differences between survival curves for each risk factor were analyzed by log-rank test. The relative risk of mortality was assessed using standardized mortality ratios (SMRs)., Results: There was no difference in the mortality rates of patients with autoimmune rheumatic diseases with or without detectable anti-Ro antibody (SMR: 1.373, 95% CI: 0.539-2.791). Six patients (4 with systemic lupus erythematosus [SLE] and two with Sjögren's syndrome [SS]) died during the follow-up period. In the whole study population, the mortality rate of patients with lymphopenia was higher than those without lymphopenia (P = 0.023). In a sub-group of patients with both SLE and SS, Kaplan-Meier analysis showed that lymphopenia and interstitial lung disease were associated with increased mortality (P = 0.024 and P = 0.023, respectively)., Conclusion: This study demonstrated that presence of the anti-Ro antibody was not associated with increased mortality in patients with autoimmune rheumatic disease. Conversely, we found that lymphopenia was independently associated with mortality in patients with autoimmune rheumatic disease., (© 2018 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Objective: Systemic autoimmune rheumatic diseases (SARD) are associated with an increased risk of premature cardiovascular disease (CVD) and all-cause mortality. We examined the potential survival benefit of statin use among patients with SARD in a general population setting., Methods: We conducted an incident user cohort study using a UK general population database. Our population included patients with a SARD as determined by Read code diagnoses of systemic lupus erythematosus, systemic sclerosis, Sjögren syndrome, dermatomyositis, polymyositis, mixed connective tissue disease, Behçet disease, or antineutrophil cytoplasmic antibodies-associated vasculitis between January 1, 2000, and December 31, 2014. We compared propensity score-matched cohorts of statin initiators and noninitiators within 1-year cohort accrual blocks to account for potential confounders, including disease duration, body mass index, lifestyle factors, comorbidities, and medication use., Results: Of 2305 statin initiators, 298 died during the followup period (mean 5.1 yrs), whereas among 2305 propensity score-matched noninitiators, 338 died during the followup period (mean 4.8 yrs). This corresponded to mortality rates of 25.4/1000 and 30.3/1000 person-years, respectively. Statin initiation was associated with reduced all-cause mortality (HR 0.84, 95% CI 0.72-0.98). When we compared the unmatched cohorts, the statin initiators (n = 2863) showed increased mortality (HR 1.85, 95% CI 1.58-2.16) compared with noninitiators (n = 2863 randomly selected within 1-year cohort accrual blocks) because of confounding by indication., Conclusion: In this general population-based study, statin initiation was shown to reduce overall mortality in patients with SARD after adjusting for relevant determinates of CVD risk.

Objectives: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in adults with systemic autoimmune rheumatic diseases (ARDs). The aim of this study was to determine whether adults with ARDs and PAH on right-sided heart catheterization (ARD-PAH) have increased mortality following lung transplantation compared with those with PAH not due to an ARD., Methods: We conducted a retrospective cohort study of 93 adults with ARD-PAH and 222 adults with PAH who underwent lung transplantation in the USA between 4 May 2005 and 9 March 2015 using data from the United Network for Organ Sharing. We examined associations between diagnosis and survival after lung transplantation using stratified Cox models adjusted for potential confounding recipient factors., Results: Among adults undergoing lung transplantation in the USA, we did not detect a difference in the multivariable-adjusted mortality rate between those with ARD-PAH and those with PAH [hazard ratio 0.75 (95% CI 0.47, 1.19)]., Conclusion: The presence of an ARD was not associated with increased mortality after lung transplantation in adults with PAH.

Objectives: Patients with rheumatic disease (RD) have an increased mortality risk compared with the general population, mainly due to cardiovascular disease (CVD). We aimed to identify patients at high risk of CVD and mortality by comparing three screening tools suitable for clinical practice., Method: In this prospective, single-centre study, consecutive patients with rheumatoid arthritis (RA), systemic autoimmune disease (SAI), or spondyloarthritides (SpA) including psoriatic arthritis underwent a comprehensive cardiovascular risk assessment. Patients were predefined as being at high risk for cardiovascular events or death if any of the following were present: European Systematic COronary Risk Evaluation (SCORE) ≥ 3%, N-terminal pro-brain natriuretic peptide (NT-proBNP) ≥ 200 pg/mL, or any pathological electrocardiogram pattern., Results: The patient population (n = 764) comprised 352 patients with RA, 260 with SAI, and 152 with SpA. After a median follow-up of 5.2 years, 6.0% of RD patients had died (7.0%, 7.2%, and 1.4% of patients in the RA, SAI, and SpA subgroups), and 5.0% had experienced a cardiovascular event (5.0%, 6.4%, and 2.8%, respectively). For all RD patients and the RA and SAI subgroups, NT-proBNP ≥ 200 pg/mL and SCORE ≥ 3% identified patients with a 3.5-5-fold increased risk of all-cause death and cardiovascular events. Electrocardiogram pathology was associated with increased mortality risk, but not with cardiovascular events., Conclusion: NT-proBNP ≥ 200 pg/mL or SCORE ≥ 3% identifies RA and SAI patients with increased risk of cardiovascular events and death. Both tools are suitable as easy screening tools in daily practice to identify patients at risk for further diagnostics and closer long-term follow-up.

Background: Indigenous populations of Canada, America, Australia, and New Zealand have increased rates and severity of rheumatic disease. Our objective was to summarize mortality outcomes and explore disease and social factors related to mortality., Methods: A systematic search was performed in medical (Medline, EMBASE, and CINAHL), Indigenous and conference abstract databases (to June 2015) combining search terms for Indigenous populations and rheumatic diseases. Studies were included if they reported measures of mortality (crude frequency, mortality rate, survival, and potential years of life lost (PYLL)) in Indigenous populations from the four countries., Results: Of 5269 titles and abstracts identified, 504 underwent full-text review and 12 were included. No studies from New Zealand were found. In five Canadian studies of systemic lupus erythematosus (SLE) patients, First Nations ethnicity was associated with lower survival after adjusting for disease and social factors, and an increased frequency of death from lupus and its complications compared to Caucasians was found. All-cause mortality was higher in Native Americans (n = 2 studies) relative to Whites with SLE after adjusting for disease and social factors, but not in those with lupus nephritis alone. Australian Aborigines with SLE frequently developed infection and lupus complications leading to death (n = 3 studies). Mortality rates were increased in Pima Indians in the United States with rheumatoid arthritis (RA) compared to those without RA. One study in Native Americans with scleroderma found nearly all deaths were related to progressive disease., Conclusions: Canadian and American Indigenous populations with SLE have increased mortality rates compared to Caucasian populations. Mortality in Canadian and Australian Indigenous populations with SLE, and in Native American populations with RA and scleroderma, is frequently attributed to disease progression or complications. The proportional attribution of rheumatic disease severity and social factors to mortality and complications leading to death between Indigenous and non-Indigenous populations has not been fully evaluated., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background and Objective: Very little is known about the outcome of patients with inflammatory rheumatic diseases in intensive care units (ICU). This retrospective study investigated the results of intensive medical treatment in these patients and the reliability of scoring systems used for the prediction of survival., Material and Methods: A case group consisting of 50 patients suffering from inflammatory rheumatic diseases was generated by analysis of patient records from the ICU at the University Hospital for Internal Medicine in Halle (Saale) in the years 2001-2010. The APACHE II score and SAPS II were used to estimate the probable mortality rate. The data were compared to those of a control group consisting of 72 patients treated on the ICU and suffering from non-inflammatory joint diseases., Results: In the case group a higher mortality rate (38 % vs. 20.8 %) and a higher frequency of respiratory, nephrogenic and cardiovascular complications were observed. In addition, these patients more often underwent artificial ventilation (66 % vs. 35 %) and had a higher rate of infections (74 % vs. 40.3 %) compared to the control group. In patients with inflammatory rheumatic diseases the SAPS II was not useful for correctly predicting mortality, whereas the APACHE II score showed sufficient agreement with the actual mortality rate., Conclusion: Patients with inflammatory rheumatic diseases displayed a poorer outcome compared with the control group in the course of the intensive care treatment. Universally applied scoring systems used to predict mortality are of limited value in this patient population.

Introduction: Multidisciplinary rehabilitation has beneficial effects on health-related quality of life (HRQoL) in patients with chronic rheumatic diseases. However, whether this intervention benefits different age groups in women or men is largely unknown., Purpose: To investigate HRQoL in patients with chronic rheumatic disease after completion of a 3-week multidisciplinary treatment, with special focus on differences in effect between age and gender groups., Method: HRQoL was measured with SF-36. Mean scores for all SF-36 domains were compared before and after the 3-week regimen and again at 3-, 6-, and 12-month follow-ups. Multivariable linear regression models using generalized estimating equations to account for repeated measurement were employed. A weighting procedure to account for differential dropouts was applied., Results: Three hundred fifty-six women and 74 men with chronic rheumatic disease were included. There were short-term improvements in all SF-36 domains irrespective of age or gender. These effects persisted for up to 1 year in the psychological, social, and energy domains for women under 50. We found no lasting effects for men; however, young men showed similar trends., Conclusion: Inpatient multidisciplinary rehabilitation improves short-term HRQoL in all patients. Younger women maintain these beneficial effects for up to 1 year. Additional intervention should be considered for elderly women and for men in order to sustain rehabilitation effects.

Variable mortality rates have been reported for patients with rheumatic diseases admitted to an intensive care unit (ICU). Due to the absence of appropriate control groups in previous studies, it is not known whether the presence of a rheumatic disease constitutes a risk factor. Moreover, the accuracy of the Acute Physiology and Chronic Health Evaluation II (APACHE II) score for predicting outcome in this group of patients has been questioned. The primary goal of this study was to compare outcome of patients with rheumatic diseases admitted to a medical ICU to those of controls. The records of all patients admitted between 1 April 2003 and 30 June 2014 (n=4020) were screened for the presence of a rheumatic disease during admission (n=138). The diagnosis of a rheumatic disease was by standard criteria for these conditions. An age- and gender-matched control group of patients without a rheumatic disease was extracted from the patient population in the database during the same period (n=831). Mortality in ICU, in hospital and after 180 days did not differ significantly between patients with and without rheumatic diseases. There was no difference in the performance of the APACHE II score for predicting outcome in patients with rheumatic diseases and controls. This score, as well as a requirement for the use of inotropes or vasopressors, accurately predicted hospital mortality in the group of patients with rheumatic diseases. In conclusion, patients with a rheumatic condition admitted to intensive care do not do significantly worse than patients without such a disease.

In recent years, multimorbidity in rheumatic conditions has gained increasing attention. Rheumatologist care for an aging patient population with multiple diseases, therefore multimorbidity is the rule, not the exception. Owing to the high prevalence and the potential interaction of coexisting diseases, multimorbidity needs to be taken into account when treating patients with chronic inflammatory conditions. In this review we address the most prevalent comorbidities in patients with rheumatic conditions and their impact on important outcomes, such as physical function, quality of life, and mortality., Competing Interests: Conflict of interestH. Radner declares that she has no competing interests.

A total of 26,203 of the deaths in Jerez de los Caballeros (Badajoz) during the 19th century were collected and grouped according to the Bertillon's Classification, in order to study the causes of death from rheumatic diseases. An analysis was made using the Death Registers, those located in the Parish Archives, and files of the Municipal Archives. There were a total of 31 deaths due to rheumatic diseases, with the 65-74 years age group being most frequent. The lack of records may be due to the inaccuracy of the diagnoses. September was the month of increased mortality., (Copyright © 2014 Elsevier España, S.L.U. y Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Background: Patients with systemic rheumatic diseases (SRDs) may require ICU management for SRD exacerbation or treatment-related infections or toxicities., Methods: This was an observational study at 10 university-affiliated ICUs in France. Consecutive patients with SRDs were included. Determinants of ICU mortality were identified through multivariable logistic analysis., Results: Three hundred sixty-three patients (65.3% women; median age, 59 years [interquartile range, 42-70 years]) accounted for 381 admissions. Connective tissue disease (primarily systemic lupus erythematosus) accounted for 66.1% of SRDs and systemic vasculitides for 26.2% (chiefly antineutrophil cytoplasm antibodies-associated vasculitides). SRDs were newly diagnosed in 43 cases (11.3%). Direct admission to the ICU occurred in 143 cases (37.9%). Reasons for ICU admissions were infection (39.9%), SRD exacerbation (34.4%), toxicity (5.8%), or miscellaneous (19.9%). Respiratory involvement was the leading cause of admission (56.8%), followed by shock (41.5%) and acute kidney injury (42.2%). Median Sequential Organ Failure Assessment (SOFA) score on day 1 was 5 (3-8). Mechanical ventilation was required in 57% of cases, vasopressors in 33.9%, and renal replacement therapy in 28.1%. ICU mortality rate was 21.0% (80 deaths). Factors associated with ICU mortality were shock (OR, 3.77; 95% CI, 1.93-7.36), SOFA score at day 1 (OR, 1.19; 95% CI, 1.10-1.30), and direct admission (OR, 0.52; 95% CI, 0.28-0.97). Neither comorbidities nor SRD characteristics were associated with survival., Conclusions: In patients with SRDs, critical care management is mostly needed only in patients with a previously known SRD; however, diagnosis can be made in the ICU for 12% of patients. Infection and SRD exacerbation account for more than two-thirds of these situations, both targeting chiefly the lungs. Direct admission to the ICU may improve outcomes.

Background: Cardiovascular comorbidities are common in rheumatic diseases and are associated with an increased mortality risk but have not been studied in a working population, with less severe disease. Also, the impact of premature cardiovascular mortality on work participation has been neglected until now., Objectives: The objectives of this study were to evaluate the cardiovascular risk in working individuals with inflammatory rheumatic diseases and to explore whether cardiovascular morbidity and mortality are associated with decreased work participation in this population., Methods: Employees from 45 companies in The Netherlands (n = 12,140) were prospectively followed up from 1998 onward by annual questionnaires. Data covering 10 years of follow-up was available (1998-2008) for rheumatic and cardiovascular morbidities. Self-reported rheumatic and cardiovascular diseases were verified by clinical review in hospital records in a subsample living in 1 specific region of The Netherlands. Information on the vital status was obtained by linking our records to national registries. Cox proportional hazards models were used to determine the contribution of cardiovascular comorbidity on mortality, with adjustment for confounders., Results: In the sample verified by clinical review, the 10-year risk of developing cardiovascular diseases tended to be increased in workers with inflammatory rheumatic diseases (n = 17) at baseline (relative risk, 2.30; 95% confidence interval [CI], 0.91-5.81) and was significantly increased in those with gout (n = 18) at baseline (relative risk, 3.64; 95% CI, 1.64-8.09) as compared with those without inflammatory rheumatic diseases or gout, respectively. Gout (n = 31; hazard ratio, 4.19; 95% CI, 1.33-13.25) and cardiovascular diseases (n = 206; hazard ratio, 2.19; 95% CI, 1.24-3.84) were significantly related to 10-year mortality. No deaths had occurred in individuals with inflammatory rheumatic diseases during follow-up., Conclusions: In this study, gout was significantly associated with cardiovascular comorbidity and mortality, but inflammatory rheumatic diseases were not. Decreased work participation in workers with gout and potentially inflammatory rheumatic diseases can be expected because of an increased morbidity but not mortality risk.

Objective: There is no consensus on which comorbidity index is optimal for rheumatic health outcomes research. We compared a new Rheumatic Disease Comorbidity Index (RDCI) with the Charlson-Deyo Index (CDI), Functional Comorbidity Index (FCI), Elixhauser Total Score (ETS), Elixhauser Point System (EPS), and a simple comorbidity count (COUNT) using a US cohort of rheumatoid arthritis (RA) patients., Methods: Using administrative diagnostic codes and patient self-reporting, we tested predictive values of the RDCI, CDI, FCI, ETS, EPS, and COUNT for 2 outcomes: all-cause mortality and physical functioning. Indices were compared using 3 models: bare (consisting of age, sex, and race), administrative (bare plus visit frequency, body mass index, and treatments), and clinic (administrative plus erythrocyte sedimentation rate, nodules, rheumatoid factor positivity, and patient activity scale)., Results: The ETS and RDCI best predicted death, with FCI performing the worst. The FCI best predicted function, with ETS and RDCI performing nearly as well. CDI predicted function poorly. The order of indices remained relatively unchanged in the different models, though the magnitude of improvement in Akaike's information criterion decreased in the administrative and clinic models., Conclusion: The RDCI and ETS are excellent indices as a means of accounting for comorbid illness when the RA-related outcomes of death and physical functioning are studied using administrative data. The RDCI is a versatile index and appears to perform well with self-report data as well as administrative data. Further studies are warranted to compare these indices using other outcomes in diverse study populations., (© 2015, American College of Rheumatology.)

Background: Inflammatory rheumatic diseases and their treatment cause various renal manifestations requiring modification of treatment., Objectives: Discussion of renal manifestations in selected rheumatic diseases, including their impact on general prognosis and therapy., Materials and Methods: Basic literature and expert opinions are analyzed and discussed., Results: Inflammatory rheumatic diseases and their treatment cause various renal manifestations, including glomerular, tubular, interstitial, and vascular damage. The type of damage determines both, associated clinical symptoms (i.e. hematuria, proteinuria, loss of kidney function) and the renal and overall survival as will be discussed here for rheumatoid arthritis, systemic lupus erythematosus, scleroderma, Sjögrens syndrome, cryoglobulinemia and ANCA-associated vasculitis., Conclusion: Renal manifestations are generally indicators of high disease activity and usually require more intensive treatment of the underlying rheumatic disease. Early and rigorous treatment, which has to be adapted to renal function, is capable of improving renal and overall survival in many of the affected patients.

Introduction: It is now well-recognised that patients with autoimmune rheumatic disease (AIRD) have a predisposition to cardiovascular disease that results in increased morbidity and mortality. Following myocardial infarction (MI), patients with rheumatoid arthritis have been shown to have an increased case fatality rate; however, this has not been demonstrated in other forms of AIRD. The aim of this study was to compare case fatality rates following a first MI in patients with AIRD versus the general population. The secondary aim was to compare revascularisation treatment following MI in patients with AIRD versus the general population., Methods: A retrospective cohort study using two population-based linked databases was undertaken. Cases of first MI from July 2001 to June 2007 were identified based on International Statistical Classification of Diseases and Related Health Problems, Tenth Revision, Australian Modification, codes. Thirty-day and one-year mortality rates were calculated (all-cause and cardiovascular causes of death). Logistic regression models were fitted to calculate the odds of mortality by AIRD status with adjustment for relevant characteristics., Results: There were 79,390 individuals with a first MI, of whom 1,409 (1.8%) had AIRD. After adjusting for relevant covariates, the odds ratio (OR) for 30-day cardiovascular mortality in patients with AIRD was 1.44 (95% confidence interval (CI): 1.25 to 1.66), and the OR for 12-month cardiovascular mortality was 1.71 (95% CI: 1.51 to 1.94). The 90-day adjusted odds of percutaneous transluminal coronary angioplasty and coronary artery bypass graft were significantly lower in the AIRD group compared with controls (OR: 0.81, 95% CI: 0.70 to 0.94, and OR: 0.52, 95% CI: 0.39 to 0.69, respectively)., Conclusions: We identified a higher risk-adjusted mortality rate for the majority of patients with AIRD at 30 days and 12 months after first MI. We also identified lower post-MI revascularisation rates in the AIRD group, suggesting there may be current gaps in cardiovascular treatment for patients with AIRD.

Objective: To assess the outcomes in a large cohort of patients suffering from rheumatic diseases admitted to the ICU of a tertiary university medical center., Methods: A retrospective chart analysis was performed in 108 patients suffering from various rheumatic diseases and the outcomes, including morbidity and mortality, were assessed in relation to the underlying diseases, treatments and complications., Results: Overall, 48 patients with rheumatoid arthritis, five patients with spondyloarthritis, 14 patients with vasculitis, 30 patients with connective tissue diseases and 11 patients suffering from other rheumatologic conditions were admitted to the intensive care unit (ICU). The reasons for ICU admission included infection (30%), cardiovascular complications (22%), gastrointestinal problems (18%), endocrinological disorders (7%), neurological complications (2%) and others (3%). A total of 4% of the admitted patients required close monitoring and 14% suffered from acute exacerbation of the underlying rheumatic disease. The ICU mortality rate was 16%, whereas the overall hospital mortality rate was 20%. Fatal outcomes were related to exacerbation of the rheumatic disease in 14% of the patients, infectious complications in 46% of the patients and other reasons in 41% of the patients. An increased Apache II score, the need for mechanical ventilation, renal replacement therapy, treatment with vasopressor drugs and plasma exchange therapy were identified as risk factors for mortality., Conclusion: The overall outcomes of critically ill patients with rheumatic diseases are impaired compared to that observed in other patient groups. However, there were no significant differences in outcomes between the different rheumatic disease groups or based on the use of immunosuppressive therapy in this study. An increased Apache II score, the need for mechanical ventilation, renal replacement therapy, treatment with vasopressor drugs and plasma exchange therapy were identified as risk factors for mortality.

Objective: To describe the frequency and types of reported adverse events and system improvement recommendations in the Morbidity and Mortality Conference (M&MC) within the Division of Rheumatology at The Hospital for Sick Children, Toronto, Ontario, Canada (SickKids)., Methods: A 5-year retrospective review of the M&MC within the Division of Rheumatology at SickKids was completed. Descriptive data including the number and types of events reported were collected. Events were categorized using an adaptation of the National Coordinating Council for Medication Error Reporting and Prevention Index. Recommendations were classified according to the Institute for Safe Medication Practices Canada., Results: Between January 2007 and December 2011, 30 regularly scheduled M&MC were held. Eighty-three cases were reviewed. The most common types of reported events were related to "miscommunication" (34.9%), "treatment/test/procedure" (22.9%), "adverse drug reactions" (12.0%), and "medication errors" (8.4%). Category A events ("an event that has the capacity to cause error") were the most common with 39.8% of the cases, followed by Category C events ("an event occurred that reached the patient, but did not cause harm") with 28.9%. Eighty-nine recommendations were made. Over half of these were classified as "information" (58.4%), followed by 11 "rules and policies" recommendations (12.4%). Of the 36 action items generated from these recommendations, most are either complete or ongoing., Conclusion: The M&MC within the Division of Rheumatology reviews a variety of events. Increased reporting of adverse events can lead to system improvements, and has the potential to improve and promote safer healthcare.