Citations

ObjectiveTo estimate the annual incidence, prevalence, and mortality of antineutrophil cytoplasmic autoantibody (ANCA)–associated vasculitis (AAV) and its subsets, granulomatosis with polyangiitis (Wegener's) (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (Churg‐Strauss) (EGPA), in a US‐based adult population.MethodsAll medical records of patients with a diagnosis of, or suspicion of having, AAV in Olmsted County, Minnesota from January 1, 1996 to December 31, 2015 were reviewed. AAV incidence rates were age‐ and sex‐adjusted to the 2010 US white population. Age‐ and sex‐adjusted prevalence of AAV was calculated on January 1, 2015. Survival rates observed in the study cohort were compared with expected rates in the Minnesota population.ResultsOf the 58 incident cases of AAV in Olmsted County during the study period, 23 (40%) were cases of GPA, 28 (48%) were cases of MPA, and 7 (12%) were cases of EGPA. Overall, 28 (48%) of the patients with AAV were women and 57 (98%) were white. The mean ± SD age at diagnosis was 61.1 ± 16.5 years. Thirty‐four patients (61%) had myeloperoxidase (MPO)–ANCAs, and 17 (30%) were positive for proteinase 3 (PR3)–ANCAs; 5 (9%) were ANCA‐negative. The annual incidence of AAV was 3.3 per 100,000 population (95% confidence interval [95% CI] 2.4–4.1). The incidence rates of GPA, MPA, and EGPA were 1.3 (95% CI 0.8–1.8), 1.6 (95% CI 1.0–2.2), and 0.4 (95% CI 0.1–0.6), respectively. The overall prevalence of AAV was 42.1 per 100,000 (95% CI 29.6–54.6). The mortality rate among AAV patients overall, and among patients with EGPA, those with MPA, and those with MPO‐ANCAs, was increased in comparison to the Minnesota general population (each P < 0.05), whereas mortality rates among patients with GPA, those with PR3‐ANCAs, and ANCA‐negative patients did not differ from that in the general population.ConclusionThe annual incidence of AAV in Olmsted County, Minnesota over the 20 years of the study was 3.3 per 100,000, with a prevalence of 42.1 per 100,000, which is substantially higher than the rates reported in other areas worldwide. The incidence of GPA was similar to that of MPA. Patients with MPA and those with EGPA, but not patients with GPA, experienced higher rates of mortality than that in the Minnesota general population. MPO‐ANCAs were a marker of poor survival in this population of patients with AAV.

ObjectiveValvular heart disease is common in patients with rheumatoid arthritis (RA). However, there is uncertainty about how often to perform echocardiographic surveillance in this population. The objective of this study was to assess the progression rate of mild and moderate aortic stenosis (AS) in patients with RA.MethodsA population‐based cohort of patients with RA and either mild (2.0–2.9 m/second) or moderate (3.0–3.9 m/second) AS was identified. Demographic, clinical, and echocardiographic data were collected. Annual progression rate of AS was then calculated for the study cohort and the impact of pertinent RA variables on progression rate determined.ResultsSixty‐eight patients with RA and mild or moderate AS met the inclusion requirements. Peak aortic valve (AV) velocity and mean AV gradient increased during the study period, whereas AV area decreased, consistent with progression of AS (P<.001). Mean (SD) annual increase in peak AV jet velocity was 0.05 m/second (0.01) and in mean AV gradient was 1.0 mm Hg (0.18). Mean annual decrease in AV area was 0.04 (0.01) cm2. The progression rate of AS was higher in patients with increased erythrocyte sedimentation rates (ESR) (P=.001).ConclusionsThe rate of AS progression in the RA population was higher in patients with increased ESR but less than that of the reported rate of AS progression in the general population. Although the cause for this finding is uncertain, these results suggest that patients with RA who have mild or moderate AS should undergo echocardiographic surveillance for disease progression similar to that of the general population.

ObjectiveTo investigate the use of glucocorticoids (GCs) and related adverse events (AEs) in a long‐term, geographically defined cohort of patients with polymyalgia rheumatica (PMR).MethodsUsing a population‐based inception cohort, details of GC therapy were abstracted from medical records of all patients diagnosed with PMR in 2000–2014. Age‐ and sex‐matched comparators without PMR were identified from the same underlying population. Cumulative and daily dosage of GC, rate of disease relapse, occurrence of GC‐related AEs, and rate of GC discontinuation were analyzed.ResultsThe study included 359 patients with PMR and 359 comparators. The median time to taper below 5 mg/day for 6 months was 1.44 years (95% confidence interval [95% CI] 1.36–1.62), while the median time to permanent discontinuation was 5.95 years (95% CI 3.37–8.88). The mean ± SD cumulative dose of GC at 2 and 5 years was 4.0 ± 3.5 grams and 6.3 ± 9.8 grams, respectively. The mean ± SD daily dose of GC at 2 and 5 years was 6.1 ± 7.6 mg/day and 7.2 ± 9.5 mg/day, respectively. There were no differences in rates of AEs between patients with PMR and comparators for diabetes mellitus, hypertension, hyperlipidemia, or hip, vertebral, or Colles fractures (P > 0.2 for all). Cataracts were more common in patients with PMR than comparators (hazard ratio 1.72 [95% CI 1.23–2.41]).ConclusionRelapse rates in PMR are highest in the early stages of therapy. Despite often protracted therapy, with the exception of cataracts, the rates of studied morbidities linked to GC are not more common in PMR than comparators.

284 Background: MM is an uncommon cancer with annual incidence of only 27,000 cases in the US. Our study determined the extent to which the number of MM patients treated annually in a treatment facility affected all-cause mortality. Methods: We used the National Cancer Data Base (NCDB) to identify adult patients with MM diagnosed from 2003-2011. NCDB is sourced from over 1,500 Commission on Cancer-accredited cancer registries representing 70% of cancer cases in the US. We classified treatment facilities by quartiles based on facility volume (mean patients/year): Quartile 1 (Q1: < 3.6), Quartile 2 (Q2: 3.6-6.1), Quartile 3 (Q3: 6.1-10.3) and Quartile 4 (Q4: > 10.3). We used hot deck imputation to account for missing data, Cox regression to analyze the association between facility volume and time-to-death, and random intercepts to adjust for multiple patients per facility. Results: There were 94,722 MM patients cared for at 1,333 facilities. Most patients (73.5%) were diagnosed and treated in the same facility. The median age at diagnosis was 67 years and 54.7% were males. The median annual facility volume was 6.1 patients/year (IQR: 3.6-10.3; range: 0.2-109.9). The distribution of patients according to facility volume was Q1 (5.2%), Q2 (12.6%), Q3 (21.9%) and Q4 (60.3%). The unadjusted median overall survival by facility volume was: Q1: 26.9 months, Q2: 29.1 months, Q3: 31.9 months and Q4: 49.1 months. After multivariable analysis adjusting for demographic (sex, age, race, ethnicity), socioeconomic (income, education, insurance type), geographic (area of residence, treatment facility location), co-morbidity (Charlson-Deyo score), and disease-specific (year of diagnosis) factors, we show that facility volume remains an independent predictor of all-cause mortality. Compared to patients treated at Q4 facilities, patients treated at lower quartile facilities had a higher risk of death (Q3 HR: 1.16 [95% CI, 1.12-1.20]; Q2: 1.21 [1.17-1.26]; Q1: 1.27 [1.22-1.34]). We observed an inverse volume-outcome relationship up to an annual facility volume of approximately 60 patients. Conclusions: MM patients treated at higher volume facilities had lower risk of mortality compared to those treated at lower volume facilities.

Purpose To determine the association between the number of patients with multiple myeloma (MM) treated annually at a treatment facility (volume) and all-cause mortality (outcome). Methods Using the National Cancer Database, we identified patients diagnosed with MM between 2003 and 2011. We classified the facilities by quartiles (Q; mean patients with MM treated per year): Q1: < 3.6; Q2: 3.6 to 6.1, Q3: 6.1 to 10.3, and Q4: > 10.3. We used random intercepts to account for clustering of patients within facilities and Cox regression to determine the volume-outcome relationship, adjusting for demographic (sex, age, race, ethnicity), socioeconomic (income, education, insurance type), geographic (area of residence, treatment facility location, travel distance), and comorbid (Charlson-Deyo score) factors and year of diagnosis. Results There were 94,722 patients with MM treated at 1,333 facilities. The median age at diagnosis was 67 years, and 54.7% were men. The median annual facility volume was 6.1 patients per year (range, 0.2 to 109.9). The distribution of patients according to facility volume was: Q1: 5.2%, Q2: 12.6%, Q3: 21.9%, and Q4: 60.3%. The unadjusted median overall survival by facility volume was: Q1: 26.9 months, Q2: 29.1 months, Q3: 31.9 months, and Q4: 49.1 months ( P < .001). Multivariable analysis showed that facility volume was independently associated with all-cause mortality. Compared with patients treated at Q4 facilities, patients treated at lower-quartile facilities had a higher risk of death (Q3 hazard ratio [HR], 1.12 [95% CI, 1.08 to 1.16]; Q2 HR, 1.17 [95% CI, 1.12 to 1.21]; Q1 HR, 1.22 [95% CI, 1.17 to 1.28]). Conclusion Patients who were treated for MM at higher-volume facilities had a lower risk of mortality compared with those who were treated at lower-volume facilities.

Abstract Rationale Patients with sarcoidosis may have an increased risk of infection similar to other immune-mediated disorders. However, the data are still limited. Objectives To investigate the risk of hospitalized infection among patients with sarcoidosis, using a population-based cohort. Methods Using the Rochester Epidemiology Project record-linkage system, a cohort of incident cases of sarcoidosis in Olmsted County, Minnesota from 1976 to 2013 was identified. Diagnosis was confirmed by individual medical record review. For each patient with sarcoidosis, a sex- and age-matched comparator without sarcoidosis was randomly selected from the same population. Medical records of cases and comparators were individually reviewed for hospitalized infection that occurred after the index date. The cumulative incidence of hospitalized infection overall and by type of infection, adjusted for the competing risk of death, was estimated. Cox models were used to compare the rate of first hospitalized infection between cases and comparators and to evaluate the association between use of immunosuppressive agents and hospitalized infection among cases. Results Three hundred and forty-five cases and 345 comparators were identified. Patients with sarcoidosis had a higher risk of a hospitalized infection with a hazard ratio (HR) of 2.00 (95% confidence interval [CI], 1.41–2.84), adjusted for age, sex, and calendar year of index date. Use of oral glucocorticoids was a significant predictor of hospitalized infection with an HR of 3.03 (95% CI, 1.33–6.90) for oral glucocorticoids not exceeding 10 mg/day and an HR of 4.48 (95% CI, 1.54–13.03) for oral glucocorticoids greater than 10 mg/day. Conclusions Patients with sarcoidosis are at increased risk of hospitalized infection. Glucocorticoid therapy is strongly associated with this increased risk.