Mortality and causes of death across the systemic connective tissue diseases and the primary systemic vasculitides.

Objectives: Studies assessing relative mortality risks across the spectrum of systemic inflammatory rheumatic diseases are largely missing. In this study, we wanted to estimate standard mortality ratios (SMRs) and causes of death in an ethnically homogeneous cohort covering all major CTDs and primary systemic vasculitides (PSVs). Methods: We prospectively followed all incident CTD and PSV cases included in the Norwegian CTD and vasculitis registry (NOSVAR) between 1999 and 2015. Fifteen controls for each patient matched for sex and age were randomly drawn from the Norwegian National Population Registry. Causes of death were obtained from the National Cause of Death Register, death certificates and hospital charts. Results: The cohort included 2140 patients (1534 with CTD, 606 with PSV). During a mean follow-up time of 9 years, 279 of the patients (13%) died, compared with 2864 of 32 086 (9%) controls (P < 0.001). Ten years after diagnosis, the lowest survival was 60% in dcSSc, 73% in anti-synthetase syndrome (ASS) and 75% in lcSSc. In the CTD group, the highest SMRs were observed in dcSSc (SMR 5.8) and ASS (SMR 4.1). In the PSV group, Takayasu arteritis (SMR 2.5) and ANCA-associated vasculitis (SMR 1.5) had the highest SMRs. Major causes of death were cardiovascular disease (CTD 27%, PSV 28%), neoplasms (CTD 25%, PSV 27%), chronic respiratory disease (CTD 20%, PSV10%) and infections (CTD 9%, PSV 16%). Conclusion: We observed premature deaths across the spectrum of CTDs and PSVs, with highest SMRs in dcSSc and ASS. The overall mortality was highest in the CTD group.

Frequencies of borderline pulmonary hypertension before and after the DETECT algorithm: results from a prospective systemic sclerosis cohort.

Objective: The DETECT algorithm was developed for screening patients with SSc at high risk of pulmonary arterial hypertension (PAH). We evaluated the impact of this algorithm in a SSc population. Methods: Patients from the unselected, prospective Oslo University Hospital SSc study were divided into the Early and DETECT cohorts, respectively, depending on whether an incident right heart catheterization (RHC) was performed before (2009-13) or after (2014-17) the DETECT algorithm was instituted. A PAH diagnosis and patient risk stratification (low, intermediate and high risk) were performed according to 2015 European Society of Cardiology guidelines. Results: At the time of the incident RHC, PAH frequency was similar between the DETECT (15/84 with PAH; 18%) and Early (16/77; 21%) cohorts, but more patients had borderline pulmonary hypertension (PH) in the DETECT (31%) than in the Early (17%) cohort. PAH risk levels were distributed differently. In the DETECT cohort, 27% and 27% were at low and high risk, respectively, at the time of PAH diagnosis. In the Early cohort, 19 and 44% were at low and high risk, respectively. A follow-up RHC, performed after [mean (SD)] 2.4 (1.8) years, showed that 39% of patients with borderline PH in the Early cohort had developed PAH. Conclusion: The DETECT algorithm did not alter PAH incidence in this unselected SSc population. However, it appeared to affect the risk distribution at the time of PAH diagnosis and increased the frequency of borderline PH cases. These findings may translate into novel opportunities for earlier PAH treatment and, possibly, prevention.

Successful use of rituximab in fibrillary glomerulopathy.

Fibrillary glomerulopathy (FG) can occur either alone or co-existing with other proteinuric glomerular disorders. FG has been associated with poor renal outcomes leading to End Stage Renal Disease (ESRD). Since FG is a relatively rare disorder, limited information is available concerning treatment protocols. We present two patients with FG who were treated with rituximab after they had already progressed to stage 3 chronic kidney disease (CKD) with worsening proteinuria. Rituximab therapy resulted in long-term stabilization of renal function.

Farm Injuries: Animal Most Common, Machinery Most Lethal: An NTDB Study.

To characterize both emergency room (ER) and hospital discharge dispositions of patients presenting with farm-related injuries. The 2012 National Trauma Data Bank was queried in August 2017 for injuries occurring on a farm. Patients were stratified by gender, age group, race, Injury Severity Score (ISS), and injury type. We performed logistic regression analysis to correlate parameters with likelihood of discharge home or death. P values < 0.05 were considered significant. Five thousand six hundred thirty-one patients were identified, the majority of whom were male (72%) and white (85%). The most common mechanisms of injury included animal-related (29%), followed by falls, vehicles, and other causes. The highest ISSs were seen in vehicular injuries (11% ISS of 25+) and the greatest fatality rate was seen in machinery injuries (4%). Four thousand seven hundred fifty-three (84%) patients were admitted to the hospital, and 4056 (72%) were discharged home from the ER or after hospitalization. One hundred thirty patients (2%) died of their farm-related injury. Most patients presenting to the ER with farm-related injuries survive, are admitted to the hospital, and are ultimately discharged home. Few patients die of their injuries. Animal injury is most common and machinery injury most lethal of farm trauma patients presenting to the ER.