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: 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: 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: 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: Antiphospholipid Antibody (aPL) are described in 11-40% of patients with SLE. Whether the presence of any of the aPLs or combinations thereof, can accurately predict the risk of thrombosis, non-thrombotic events and obstetric outcome in SLE has been debatable. We report the prevalence, associations and outcomes in SLE patients with aPL. Methods: From the ongoing prospective ...

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: Muscle inflammation in systemic lupus erythematosus (SLE) usually presents as generalised myalgia in the presence of active disease elsewhere and responds well to treatment. There is paucity of data on myositis in lupus. We assessed the frequency and association of myositis in an inception cohort of SLE from India (INSPIRE). Methods: The INSPIRE database includes patients sa...

Nov 13th, 2022 - Background/Purpose: Patients with systemic lupus erythematosus (SLE) had a higher risk of pericarditis, which could be life-threating, but there has been no research focusing on the prognosis of patients with lupus pericarditis. This study was conducted the prognostic factors of mortality in patients with lupus pericarditis. Methods: In this observational cohort study, SLE inpatients with lupus...

Nov 13th, 2022 - Background/Purpose: Scleroderma Renal Crisis (SRC) is a severe and life-threatening complication of Systemic Sclerosis (SSc). Given the rare nature of SSc, it is challenging to collect data to further our understanding of SRC. We sought to collect and analyze data on SRC patients to allow us to better understand this condition by discovering new insights regarding risk factors for severe outcom...

Nov 13th, 2022 - Background/Purpose: Pulmonary arterial hypertension (PAH) leads to a progressive right heart failure and death.PAH is a leading cause of death in Systemic Sclerosis (SSc). To stratify the prognosis and 1-year survival of patients with PAH REVEAL 2.0 score was developed. It classifies each patient in low, moderate and high-risk of death at the first year of follow-up. It demonstrated greater ris...

Nov 13th, 2022 - Background/Purpose: Pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) are the leading causes of systemic sclerosis (SSc) associated death. There is increasing interest in the prognosis and outcome of patients who develop both PAH and ILD. We aimed to describe the clinical phenotype and prognosis of people within the Australian Scleroderma Cohort Study (ASCS) with concurr...

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...

Nov 13th, 2022 - Background/Purpose: The long-term morbidity and mortality of childhood-onset SLE (cSLE) after transition to adult care is not well documented. The present study aims to fill this knowledge gap by analyzing outcomes in a large province-wide cSLE clinical cohort linked to multiple administrative healthcare databases. Our objectives were to: 1) determine all-cause and cause-specific mortality rate...

Nov 12th, 2022 - Background/Purpose: Medication management strategies in patients with lupus-related end stage kidney disease (ESKD) have not been studied. Our prior work has shown that HCQ may be underutilized and corticosteroids may be overutilized in these patients. We aimed to assess whether patients with lupus nephritis who continue HCQ after ESKD have lower risk of cardiac outcomes, thrombosis, infections...

Nov 12th, 2022 - Background/Purpose: Most previous studies have demonstrated a similar overall survival in patients with GCA compared to the background population. However, we have previously reported an increased mortality during the first two years after diagnosis in patients diagnosed through 2010, in particular among those aged < 70 years. Up till now, no data on cause of death has been presented from our a...