Prevalence of ANCA-associated vasculitis amid natural gas drilling sites in West Virginia.

BACKGROUND: The epidemiology of ANCA-associated vasculitis (AAV) varies by ethnicity and region. Environmental exposure has been implicated in the pathophysiology of MPO-AAV. The aim of this study was to evaluate the epidemiology of AAV and explore a potential relationship with environmental factors in north central West Virginia. METHODS: This is a retrospective cohort study of 212 patients diagnosed with AAV at West Virginia University and its affiliated hospitals from January 1, 1990 to December 31, 2019. We assessed prevalence of AAV over time according to patient's zip codes and counties. Silica exposure through natural gas extraction was considered as a possible environmental factor. RESULTS: The proportion of patients with MPO-ANCA increased after 2010 (37.5% before 2010 vs 61% after 2010, p = 0.008). At the same time, the prevalence of AAV in Monongalia and surrounding counties has increased significantly after 2010 from 64.8 to 141.9 cases per million (p = 0.001). The increase in prevalence of AAV was primarily due to an increase in MPO-AAV (43 vs 101.7 cases per million before and after 2010, respectively, p = 0.028). During this time, the production of natural gas through fracking increased, rising more than tenfold after 2010 (p-value < 0.001). Heat mapping reveals that the increase in cases of AAV occurred in areas of increased fracking activity. CONCLUSIONS: There was an increase in the prevalence of patients who were newly diagnosed with AAV over time in north central West Virginia. Further studies are required to ascertain the potential role of environmental exposure in the pathophysiology of AAV.

Antibody subtypes and titers predict clinical outcomes in ANCA-associated vasculitis.

The objective of this study is to evaluate the association between antineutrophil cytoplasmic autoantibody (ANCA) subtype and ANCA titers on clinical outcomes and disease activity among a cohort of patients from Central Appalachia diagnosed with ANCA-associated vasculitis (AAV) over a 3-decade period. This is a retrospective chart review of all patients diagnosed with AAV. ANCA subtypes (myeloperoxidase (MPO) and proteinase 3 (PR3)) and titers at the time of diagnosis and at the time of relapse or last follow-up were evaluated along with patient outcomes. Outcomes of interest included relapse, development of end-stage renal disease (ESRD) and mortality. Sensitivity analysis and multivariable analysis were performed. Of the 202 patients, 111 patients were MPO-ANCA positive and 91 patients were PR3-ANCA positive. Relapse was more frequent among patients with PR3-ANCA compared to MPO-ANCA (35% vs 12%, p < 0.001). In both ANCA subgroups, the strongest predictor of relapse was an increase in titers prior to relapse, HR 8.1 (95% CI 1.6-40), p 0.009. Patients who achieved serological remission had a lower risk of ESRD [sub-HR 0.31 (95% CI 0.11-0.89)] and mortality [HR (95% CI) 0.24 (0.07-0.7)]. PR3-ANCA was associated with higher risk of ESRD [sub-HR 3.1 (95% CI 1.1-8.5)]. There was no difference in mortality between patients with MPO-ANCA and PR3-ANCA. Our study supports the use of both ANCA subtypes and titer levels for predicting clinical outcomes in patients receiving treatment for AAV. Monitoring of ANCA antibody titers may be useful since both serological remission and increase in titers provide prognostic information.