Intravenous pulse methylprednisolone for induction of remission in severe ANCA associated Vasculitis: a multi-center retrospective cohort study.

BACKGROUND: Intravenous pulse methylprednisolone (MP) is commonly included in the management of severe ANCA associated vasculitis (AAV) despite limited evidence of benefit. We aimed to evaluate outcomes in patients who had, or had not received MP, along with standard therapy for remission induction in severe AAV. METHODS: We retrospectively studied 114 consecutive patients from five centres in Europe and the United States with a new diagnosis of severe AAV (creatinine > 500 µmol/L or dialysis dependency) and that received standard therapy (plasma exchange, cyclophosphamide and high-dose oral corticosteroids) for remission induction with or without pulse MP between 2000 and 2013. We evaluated survival, renal recovery, relapses, and adverse events over the first 12 months. RESULTS: Fifty-two patients received pulse MP in addition to standard therapy compared to 62 patients that did not. There was no difference in survival, renal recovery or relapses. Treatment with MP associated with higher risk of infection during the first 3 months (hazard ratio (HR) 2.7, 95%CI [1.4-5.3], p = 0.004) and higher incidence of diabetes (HR 6.33 [1.94-20.63], p = 0.002), after adjustment for confounding factors. CONCLUSIONS: The results of this study suggest that addition of pulse intravenous MP to standard therapy for remission induction in severe AAV may not confer clinical benefit and may be associated with more episodes of infection and higher incidence of diabetes.

Healthcare utilization and expenditures for United States Medicare beneficiaries with systemic vasculitis.

OBJECTIVE: The Medicare federal insurance program is the most common United States insurer of patients with systemic vasculitis (SV). We compared healthcare utilization and expenditures for Medicare beneficiaries with versus without SV. METHODS: This national, retrospective study used 2010 claims and enrollment data for a 100% cohort of Medicare Part A and B beneficiaries with ≥1 claim including a diagnosis for a form of SV (n = 176,498), and a randomly selected group of non-SV beneficiaries (n = 46,561). Outcomes included annual counts of events in 16 categories of medical services (e.g., inpatient stays, physician visits, tests, and imaging events), and total annual Medicare and patient medical expenditures. We used linear regression with bootstrapped standard errors to compare utilization and expenditures by SV status, before and after matching on age and sex. Prescription drug fills and expenditures for SV (n = 95,157) and non-SV (n = 24,992) beneficiaries with Part D drug benefits were also compared. RESULTS: After matching, Medicare spent $11,004 more per patient in 2010 for medical services, and $773 more on prescription drugs, for SV versus non-SV beneficiaries. SV beneficiaries spent $1547 more for medical services and $211 more for prescription drugs. Except for hospice, SV beneficiaries had greater utilization of all services, including two-to-three times more dialysis events, hospital readmissions, inpatient stays, skilled nursing facility stays, and medical tests. CONCLUSIONS: The average Medicare beneficiary with SV incurs about double the annual healthcare expenditures compared to their non-SV counterparts, attributable to increased utilization of almost all categories of care.

Trends in Long-Term Outcomes Among Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis With Renal Disease.

OBJECTIVE: It is still not clear how advances in the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have impacted long-term outcomes. We undertook this study to examine changes over 25 years in long-term clinical outcomes, including the impact of renal function at diagnosis (a potential marker of time to disease detection) and the duration of cyclophosphamide use in AAV patients with renal involvement. METHODS: We included ANCA-positive patients with biopsy-proven AAV diagnosed between 1985 and 2009 who were followed up in the Glomerular Disease Collaborative Network inception cohort. Outcomes included the composite outcome of end-stage renal disease (ESRD) or death as well as relapse. Cox proportional hazards or competing risks regression models were adjusted for potential baseline confounders. RESULTS: Data from 554 patients were included in the analysis. There was a decreasing 5-year risk of ESRD or death over time (P < 0.001 by log rank test for trend). After adjustment for baseline characteristics, the risk of relapse was similar across the time periods (P = 0.45 by test for trend). Serum creatinine level at baseline was the only significant predictor of an increased risk of ESRD or death (hazard ratio 1.11 per 1 mg/dl of serum creatinine [95% confidence interval 1.04-1.18], P = 0.002). CONCLUSION: In patients with renal disease secondary to AAV, over 25 years the risk of ESRD or death has decreased but the risk of relapse has not changed. A higher serum creatinine level at diagnosis is associated with a higher risk of ESRD or death, suggesting that earlier disease detection is potentially an important measure to improve outcomes in AAV.

Clinical characteristics and outcome of pauci-immune glomerulonephritis in African Americans.

OBJECTIVES: Pauci-immune glomerulonephritis is rare in African Americans (AA) and the clinical presentation and treatment outcomes of vasculitis have not been well described. METHODS: We identified patients who were 2-92 years of age between 1983 and 2011 with a diagnosis of biopsy-proven pauci-immune glomerulonephritis (GN) at any point during their disease course. Comparing AA to Caucasian patients, we examined demographics, clinical features at presentation, treatment and outcomes of relapse, end-stage renal disease (ESRD), and death. RESULTS: Of the 672 patients, 75 were AA with the remainder being Caucasian. Compared to Caucasians, disease onset in AA was at an earlier age (52 vs. 57 years, p = 0.05) and was more often myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) positive (71% vs. 54%, p = 0.01). AA patients had a shorter median time between onset of symptoms and biopsy compared to Caucasians [median (IQR): 0.23 (0.00, 1.22) months vs. 0.66 (0.00, 3.62) months, p = 0.003]. Median [Interquartile range (IQR)] follow-up in months was 28 (5, 52) in AA and 26 (10, 55) in Caucasian patients. Median estimated glomerular filtration rate was similar at presentation (21 vs. 22 ml/min/m(2)). Both groups had similar induction treatment regimens. There was less favorable treatment response among AA compared to Caucasians for initial treatment resistance (28% vs. 18%, p = 0.05) and complete remission (72% vs. 82%, p = 0.05). There were no differences in the number of renal relapses or number of deaths between the 2 groups. Overall, in multivariable analyses controlling for age, race, ANCA type, and entry serum creatinine, there were not differences by race in treatment response, renal relapse, ESRD, or death over the entire time of follow-up. CONCLUSIONS: AA patients with pauci-immune GN are younger and more often MPO-ANCA positive compared to Caucasians. Despite a shorter time to diagnosis for AA patients, there were no differences compared to Caucasians in treatment response, ESRD, renal relapse, or death rates by race over the entire duration of follow-up.