Batesbest3829

The mean accumulated etoposide dose was 671 mg/m

(range 300-1,050 mg/m

), as compared to 1,500 mg/m

recommended the first 8 weeks of the HLH-94/HLH-2004 protocols. One child developed neutropenic fever and another neutropenic sepsis (neutrophils 0.3x10

/L at therapy onset). Five/seven children had low percentages (<5%) circulating NK-cells prior to or in association with diagnosis; NK-cell activity was pathologically low in two/five children studied. Disease-causing variants in HLH-associated genes were not found. All children were alive at latest follow-up (2-9 years after onset); neurological symptoms had normalized in four/five affected children.

Moderately dosed etoposide may be beneficial in severe and/or refractory MAS-HLH.

Moderately dosed etoposide may be beneficial in severe and/or refractory MAS-HLH.

To evaluate the efficacy and safety of apremilast, an oral phosphodiesterase 4 inhibitor, in patients with active ankylosing spondylitis (AS).

This phase III, multicenter, double-blind, placebo-controlled study (ClinicalTrials.gov NCT01583374) randomized patients with active AS (111) to placebo, apremilast 20 mg twice daily, or apremilast 30 mg twice daily for 24 weeks, followed by a long-term extension phase (up to 5 yrs). The primary endpoint was Assessment of the Spondyloarthritis international Society 20 (ASAS20) response at Week 16. The effect of treatment on radiographic outcomes after 104 weeks was assessed using the modified Stoke Ankylosing Spondylitis Spine Score (mSASSS).

In total, 490 patients with active AS were randomized in the study (placebo n = 164; apremilast 20 mg twice daily n = 163; apremilast 30 mg twice daily n = 163). The primary endpoint of ASAS20 response at Week 16 was not met (placebo 37%; apremilast 20 mg twice daily 35%; apremilast 30 mg twice daily 33%;

= 0.44 vs placebo). At Week 104, mean (SD) changes from baseline in mSASSS were 0.83 (3.6), 0.98 (2.2), and 0.57 (1.9) in patients initially randomized to placebo, apremilast 20 mg twice daily, and apremilast 30 mg twice daily, respectively. The most frequently reported adverse events through Week 104 were diarrhea, nasopharyngitis, upper respiratory infection, and nausea.

No clinical benefit was observed with apremilast treatment in patients with active AS. The safety and tolerability of apremilast were consistent with its known profile.

No clinical benefit was observed with apremilast treatment in patients with active AS. The safety and tolerability of apremilast were consistent with its known profile.

To assess trends in incidence of cardiovascular disease (CVD) and mortality following incident CVD events in patients with rheumatoid arthritis (RA) onset in 1980- 2009 versus non-RA subjects.

We studied Olmsted County, Minnesota residents with incident RA (age ≥ 18 years, 1987 ACR criteria met in 1980-2009) and non-RA subjects from the same source population with similar age, sex and calendar year of index. All subjects were followed until death, migration, or 12/31/2016. Incident CVD events included myocardial infarction and stroke. Patients with CVD before RA incidence/index date were excluded. Cox models were used to compare incident CVD events by decade, adjusting for age, sex and CVD risk factors.

The study included 905 patients with RA and 904 non-RA subjects. Cumulative incidence of any CVD event was lower in patients with incident RA in 2000s versus 1980s. Hazard Ratio [HR] for any incident CVD 2000s versus 1980s 0.53; 95% confidence interval (CI) 0.31-0.93. selleck compound The strength of association attenuated after adjustment for anti-rheumatic medication use HR 0.64, 95%CI 0.34-1.22. Patients with RA in 2000s had no excess in CVD over non-RA subjects (HR 0.71, 95%CI0.42-1.19). Risk of death after a CVD event was somewhat lower in patients with RA after 1980s HR 0.54, 95%CI0.33-0.90 in 1990s and HR 0.68, 95%CI0.33-1.41 in 2000s versus 1980s.

Incidence of major CVD events in RA has declined in recent decades. The gap in CVD occurrence between RA patients and the general population is closing. Mortality after CVD events in RA may be improving.

Incidence of major CVD events in RA has declined in recent decades. The gap in CVD occurrence between RA patients and the general population is closing. Mortality after CVD events in RA may be improving.

To estimate the prevalence and incidence of multimorbidity (MM) in a population-based cohort of patients with rheumatoid arthritis (RA) compared to subjects without RA.

Between 1999-2013, residents of Olmsted County, Minnesota with incident RA who met the 1987 American College of Rheumatology criteria were compared to age- and sex-matched non-RA subjects from the same population. Twenty-five chronic comorbidities from a combination of the Charlson, Elixhauser, and Rheumatic Disease Comorbidity Indices were included, excluding rheumatic comorbidities. The Aalen-Johansen method was used to estimate the cumulative incidence of MM (MM2+; ≥ 2 chronic comorbidities) or substantial MM (MM5+; ≥ 5), adjusting for the competing risk of death.

The study included 597 patients with RA and 594 non-RA subjects (70% female, 90% White, mean age 55.5 yrs). At incidence/index date, the prevalence of MM2+ was higher in RA than non-RA subjects (38% RA vs 32% non-RA,

= 0.02), whereas prevalence of MM5+ was similar (5% RA vs. 4% non-RA,

= 0.68). During follow-up (median 11.6 yrs RA, 11.3 yrs non-RA), more patients with RA developed MM2+ (214 RA vs 188 non-RA; adjusted HR 1.39, 95% CI 1.14-1.69). By 10 years after RA incidence/ index, the cumulative incidence of MM2+ was 56.5% among the patients with RA (95% CI 56.5-62.3%) compared with 47.9% among the non-RA (95% CI 42.8-53.7%). Patients with RA showed no evidence of increase in incidence of MM5+ (adjusted HR 1.17, 95% CI 0.93-1.47).

Patients with RA have both a higher prevalence of MM at the time of RA incidence as well as increased incidence thereafter.

Patients with RA have both a higher prevalence of MM at the time of RA incidence as well as increased incidence thereafter.The Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA) held its annual meeting in 2020 in an online format due to travel restrictions during the coronavirus disease 2019 (COVID‑19; caused by SARS-CoV-2) pandemic. The virtual meeting was attended by 351 rheumatologists, dermatologists, representatives of biopharmaceutical companies, and patient research partners. Similar to previous years, GRAPPA's annual meeting focused on the 3 overlapping missions of education, research, and clinical care of psoriatic disease. Trainee sessions this year included the annual trainee symposium and a grant-writing workshop. Plenary sessions included updates on COVID-19 and psoriatic disease from multispecialty and patient perspectives, and updates on pustular psoriasis and associated musculoskeletal manifestations. Progress on research and updates were presented for the following groups Collaborative Research Network, Outcome Measures in Rheumatology (OMERACT) Psoriatic Arthritis Working Group, International Dermatology Outcome Measures, Composite Measures, Education Committee, and Treatment Guidelines.