Jan 17th, 2023 - Patients with anti-Jo-1 antibodies (Abs) and anti-melanoma differentiation-associated protein 5 (MDA5) Abs are at a higher risk of interstitial lung disease (ILD) and have a mortality rate higher than that of patients with anti-Jo-1 Abs. This study investigated differences in the clinical characteristics and prognosis of patients with anti-Jo-1 Abs and anti-MDA5 Abs with dermatomyositis (DM). W...

Jan 15th, 2023 - Primary myelofibrosis [PMF] is a myeloproliferative neoplasm associated with reduced overall survival (OS). Management strategies for PMF have evolved over the last two decades, including approval of ruxolitinib as the first Janus kinase 1 (JAK1)/JAK2 inhibitor for patients with intermediate or high-risk myelofibrosis. This study assessed changes in mortality before and after ruxolitinib approv...

Jan 14th, 2023 - This study aimed to assess the mortality of PsA before and during the COVID-19 pandemic. From the prospective, multicenter PsART-ID (Psoriatic Arthritis Registry-International Database), patients from Turkey were analyzed by linking the registry to the Turkish Cause of Death Registry. The outcome of interest was death from any cause, pre-pandemic (since the onset of registry-March 2014-March 20...

Jan 5th, 2023 - Between October 2007 and July 2013, 183 Mayo Clinic patients (median age 65 years; 58% males) with high/intermediate risk myelofibrosis (MF) were enrolled in consecutive phase 1/2 JAK2 inhibitor (JAKi) clinical trials with momelotinib (n = 79), ruxolitinib (n = 50), fedratinib (n = 23) and BMS-911543 (n = 31). Using conventional criteria, the respective response rates for spleen and "transfusio...

Dec 22nd, 2022 - Solid organ transplant (SOT) recipients are at increased risk for morbidity and mortality from COVID-19 due to their immunosuppressed state and reduced immunogenicity from COVID-19 mRNA vaccines. This investigation examined the association between COVID-19 mRNA vaccination status and mortality among SOT recipients diagnosed with COVID-19. A retrospective, registry-based chart review was conduct...

Dec 5th, 2022 - Heme oxygenase one (HO-1) is considered a poor prognostic factor for survival in patients with severe-to-critical coronavirus disease (COVID-19), but the clinical correlation between heme catabolism biomarkers and COVID-19-related sepsis is unknown. The etiopathogenetic hypothesis of HO-1 response during sepsis in patients with poor prognosis should be clarified. This study aimed to investigate...

Nov 22nd, 2022 - Sudden changes in health care utilization during the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic may have impacted the performance of clinical predictive models that were trained prior to the pandemic. In this study, we evaluated the performance over time of a machine learning, electronic health record-based mortality prediction algorithm currently used in clinical pra...

Nov 19th, 2022 - Previous studies suggested that ongoing treatment with renin-angiotensin-aldosterone system (RAAS) inhibitor drugs may alter the course of SARS-CoV-2 infection and promote the development of more severe forms of the disease. The authors conducted a comparative, observational study to retrospectively analyze data collected from 394 patients admitted to ICU due to SARS-CoV-2 pneumonia. The primar...

Nov 18th, 2022 - Immune-related adverse events, particularly severe toxicities such as myocarditis, are major challenges to the utility of immune checkpoint inhibitors (ICIs) in anticancer therapy1. The pathogenesis of ICI-associated myocarditis (ICI-MC) is poorly understood. Pdcd1-/-Ctla4+/- mice recapitulate clinicopathological features of ICI-MC, including myocardial T cell infiltration2. Here, using single-...

Nov 14th, 2022 - Background/Purpose: To describe the clinical characteristics of a 20-year cohort of patients with systemic autoimmune diseases (SAD) admitted to a medical intensive care unit (ICU) and to identify mortality prognostic factors. Methods: A single-center retrospective, observational study including all adult patients with SAD admitted to a medical ICU of a tertiary referral center between 1999 and...

Nov 14th, 2022 - Background/Purpose: Skeletal myopathy in systemic sclerosis (SSc) is poorly understood. The etiology of myopathy in SSc is heterogeneous and can result from an inflammation, disuse, malnutrition, or other neuromuscular diseases. The purpose of this study was to determine the clinical phenotype and risk of mortality of SSc patients with and without a myopathy. Methods: To understand the clinical...

Nov 14th, 2022 - Background/Purpose: SLE is a chronic autoimmune disorder associated with increased mortality compared to the general population; treatment for SLE has improved and survival rates have substantially improved from initial 3-year mortality rates of 40% in 19531, however, there is still a 3-fold increased risk of death2 with early causes of death from SLE activity, steroid use, and infections, whil...

Nov 14th, 2022 - Background/Purpose: To study the effect of osteoporosis on major adverse cardiovascular events (MACEs) and mortality in a longitudinal cohort of patients with systemic lupus erythematosus (SLE). Methods: Patients who fulfilled ≥4 1997 ACR criteria for SLE and participated in a cross-sectional study on osteoporosis in the year 2011 were studied. All patients had a DEXA scan performed between yea...

Nov 14th, 2022 - Background/Purpose: Multiple studies have documented that patients with SLE have a higher all-cause mortality. Active disease contributes to early deaths with later deaths more likely due to cardiovascular disease and malignancy. In this study we present the pattern of mortality in a single long-term, prospective cohort using death certificates to document cause of death. Methods: The cohort co...

Nov 14th, 2022 - Background/Purpose: Primary Sjögren Syndrome (pSS) patients present an increased risk of Non-Hodgkin lymphoma (NHL). There is no consensus on the therapeutic management of low-grade NHL. Two strategies can be proposed; either a ”wait and see” strategy or active therapeutic strategy. The objective of our study was to describe characteristics of NHL in pSS, and identify predictive and prognosis f...

Nov 13th, 2022 - Background/Purpose: Antiphospholipid syndrome (APS) is associated with diffuse alveolar hemorrhage (DAH). However, only case reports and small case series are available in the literature. We aimed to describe the clinical characteristics, therapeutic strategies, and clinical outcomes of patients with APS and DAH from a multi-center institution. Methods: We conducted a multi-center, retrospectiv...

Nov 13th, 2022 - Background/Purpose: To describe the clinical characteristics, therapies, and outcomes of patients with antiphospholipid syndrome (APS) and adrenal hemorrhage (AH). Methods: We performed a multicenter retrospective cohort study identifying patients with APS and AH, up to December 12, 2021. We included patients with APS meeting the Sydney criteria and an episode of AH, defined as the presence of ...

Nov 13th, 2022 - Background/Purpose: Scleroderma (SSc) Heart Involvement (SHI) is an under recognised complication of SSc and may manifest as arrhythmias or sudden cardiac death(SCD). The incidence of SCD in Australia is approximately 35-89 per 100,000 (1) however there is limited data in SSc to date. We sought to determine the incidence of SCD, arrhythmias and conduction abnormalities in SSc. Methods: We searc...

Nov 13th, 2022 - Background/Purpose: There is increasing evidence that rheumatoid arthritis (RA) is a risk factor for death related to COVID-19, however data on the risk of death for other inflammatory arthropathies is equivocal or non-existent. Our primary objective was to assess whether certain inflammatory rheumatic diseases (IRDs) like rheumatoid arthritis (RA), ankylosing spondylitis (AS), giant cell arter...

Nov 13th, 2022 - Background/Purpose: Several autoimmune diseases (AD) may be associated with interstitial lung disease (ILD), contributing to increased morbidity and mortality in these patients. We aimed to study the risk of ILD in different ADs and the impact of ILD on the outcomes of AD hospitalized patients utilizing the national inpatient sample database. Methods: We used the national inpatient sample of 20...