Matteson, Eric L.

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.

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.

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.