Inpatient epidemiology and economic burden of granulomatosis with polyangiitis: a 10-year study of the national inpatient sample.

OBJECTIVE: To characterize inpatient epidemiology and economic burden of granulomatosis with polyangiitis (GPA). METHODS: Patients with GPA were identified from the Nationwide Inpatient Sample (NIS), the largest inpatient database in the USA consisting of over 4000 non-federal acute care hospitals, using the ICD-9 CM code. A cohort of comparators without GPA was also constructed from the same database. Data on demographics, procedures, length of stay, mortality, morbidity and total hospitalization charges were extracted. All analysed data were extracted from the database for the years 2005-2014. RESULTS: The inpatient prevalence of GPA was 32.6 cases per 100 000 admissions. GPA itself (38.3%), pneumonia (13.7%) and sepsis (8.4%) were the most common reasons for admission. After adjusting for potential confounders, the all-cause mortality adjusted odds ratio (aOR) of patients with GPA was significantly higher than that of patients without GPA (aOR 1.20; 95% CI: 1.41, 1.61). This was also true for several morbidities, including acute kidney injury, multi-organ failure, shock and need for intensive care unit admission. Hospitalizations of patients with GPA were associated with higher cost as demonstrated by an adjusted additional mean of $5125 (95% CI: $4719, $5531) for total hospital cost and an adjusted additional mean of $16 841 (95% CI: $15 280, $18 403) for total hospitalization charges when compared with patients without GPA. CONCLUSION: Inpatient prevalence of GPA was higher than what would be expected from prevalence in the general population. Hospitalizations of patients with GPA were associated with higher morbidity, mortality and cost.

Burden of illness and costs associated with eosinophilic granulomatosis with polyangiitis: evidence from a managed care database in the United States.

BACKGROUND: Data on the clinical and economic burden of eosinophilic granulomatosis with polyangiitis (EGPA) are limited. OBJECTIVE: To assess the real-world clinical and economic outcomes of patients diagnosed with EGPA vs patients with asthma (present in > 90% of EGPA cases) receiving treatment in the United States. METHODS: This retrospective cohort study (HO-17-17742) used administrative claims data (July 1, 2007-May 31, 2017) from the Optum Research Database. Eligible patients were aged at least 18 years at index (first date that patients met the EGPA or asthma cohort definition), with a minimum of 6 months of continuous health plan coverage before the index (baseline) period and 12 months following and including the index date (follow-up period). Patients with EGPA were identified either via published algorithms using claim code combinations for conditions and medications (before October 1, 2015) or via a claim with the EGPA ICD-10-CM code (M30.1, after October 1, 2015). Patients with asthma were identified based on ICD-9-CM and ICD-10-CM diagnosis codes and at least 3 pharmacy asthmarelated medication claims within a year of diagnosis. Outcomes included all-cause health care costs (primary), all-cause health care resource utilization (HCRU), systemic corticosteroid (SCS) use, and EGPA relapses requiring hospitalization and EGPA-related (based on EGPA-related HCRU) relapses during the follow-up period (all secondary). EGPA and asthma cohorts were matched (1:3) via propensity score matching based on demographic, insurance, and index timing covariates. RESULTS: 8,904 patients were included in the matched EGPA (n = 2,226) and asthma (n = 6,678) cohorts (mean [SD] age: 59.7 [14.2] vs 59.6 [14.7] years; Quan-Charlson Comorbidity Index scores: 1.8 [1.7] vs 0.8 [1.4]). During follow-up, mean (SD) all-cause costs ($49,593 [$88,161] vs $21,122 [$40,110]; P < 0.001), all-cause HCRU (P < 0.001), and the proportion of patients with 1 or more SCS claims (72.3% vs 66.9%; P < 0.001) were significantly greater in the EGPA vs asthma cohorts, respectively. Mean daily SCS dose (43.6-45.5 mg/day) was similar between cohorts; patients with EGPA had significantly (P < 0.001) longer periods taking SCS doses at least 4 mg/day (mean [SD]: 64.9 [95.6] vs 14.6 [39.3] days) and at least 7 mg/day (52.8 [82.0] vs 12.1 [30.6] days). 35.2% (n = 784/2,226) and 44.1% (n = 981/2,226) of patients with EGPA experienced a minimum of 1 EGPA relapse requiring hospitalization, and at least 1 EGPA-related relapse, respectively. Mean (SD) total all-cause costs were greater than 3-fold higher in patients with vs without a relapse requiring hospitalization ($92,825 [$128,562] vs $26,087 [$38,082]; P < 0.001) and for patients with vs without an EGPA-related relapse ($78,081 [$120,775] vs $27,145 [$35,584]; P < 0.001). CONCLUSIONS: Patients with EGPA have more comorbidities, greater health care costs and HCRU, and use SCS more frequently than patients with asthma. Additionally, more than one third of patients with EGPA experienced disease relapses over 12 months. These results highlight the high disease burden in patients with EGPA and the need for improved treatment options. DISCLOSURES This study was funded by GlaxoSmithKline (GSK ID: HO-17-17742). Bell is an employee of GSK and holds stock/share options in GSK. Blauer-Peterson is an employee of Optum, which was funded by GSK to conduct the study. Mao was an employee of Optum at the time the study was conducted. The authors report no other potential conflicts of interest. These data have previously been presented as a poster at the American College of Rheumatology/Association of Rheumatology Health Professionals Annual Meeting, Chicago, IL, October 19-24, 2018.

Wegener's granulomatosis: survey of 701 patients in North America. Changes in outcome in the 1990s.

OBJECTIVE: To study the medical and socioeconomic impact of Wegener's granulomatosis (WG) in a large cohort (n = 701) of patients who are members of the international WG Support Group (WGSG). METHODS: Forty questions designed and validated by one of the authors and reviewed by the medical consultants of the WGSG International were mailed to 1690 patients with WG who are members of the WGSG; 701 (41%) patients returned the questions. Diagnosis of WG was self-reported for purpose of this questionnaire. Study domains included demographic features, education, analysis of categories of medical care providers, organ system involvement, delay in diagnosis, frequency and sites of biopsies to assist in diagnosis, treatment outcome, familial association, disability, and financial effect. We compared some of these features in patients whose diagnosis was made in the 1970s, 1980s, or 1990s. RESULTS: In our cohort WG was slightly more prevalent in women (56%), particularly if the disease started at a younger age (9-40 years). Peak age period at disease onset was 45-65 years. Ninety-eight percent of patients were Caucasian. Diagnosis of WG was usually made by a specialist, and the majority of patients received subsequent care by specialists. During the past decade only 7% of patients received a diagnosis of WG upon their first visit to a physician. A period of 3-12 months passed from onset of features of WG to achieving a diagnosis in the majority of patients. Compared to the period 1970-90, in recent years fewer patients had biopsies performed for diagnostic purposes. This observation correlated with increased use of antineutrophil cytoplasmic antibodies. In the 1990s the most common reported therapy was combination of corticosteroids and cyclophosphamide (73%). Patients also reported initial therapy with methotrexate (11%), trimethoprim-sulfamethoxa-zole (32%), and azathioprine (5%). Patients rarely reported other family members with WG. In none of 12 WG patients who had a twin did the twin have WG. The survey did not identify any specific environmental exposure, occupation, or hobby that was overrepresented among patients. One hundred seventy-nine WG patients reported that their disease had a significant financial impact on their lives. CONCLUSION: Information from this survey of 701 patients is consistent with physician reported data about organ involvement, initial manifestations and therapy, and outcomes in WG. More WG patients in the 1990s were diagnosed after first physician encounter. This survey did not reveal any predisposing or inducing environmental or familial factors, and showed fewer patients become disabled and more were able to work full time.