Background/Purpose:

ANCA-associated vasculitis (AAV) is a cause of multi-organ disease in all ages, but peaks at ages 65-74. Limited data is available on the characteristics of AAV in the elderly. Our aim is to compare clinical and serological presentations, treatment regimens, and survival outcomes between younger and older AAV patients.

Methods:

A retrospective review of patients younger and older than 65yrs [(<65grp); (≥65grp), respectively] diagnosed with AAV was conducted at our institution between 2005 and 2016. Data on demographics, organ system involvement, ANCA-MPO/PR3 serology, BVAS, Vasculitis Damage Index (VDI), 5 Factor Score (FFS) and treatment was recorded. Chi square tests of association and two-sample t-test for group mean differences were used to compare groups. Survival was assessed using Cox proportional hazard ratio and Kaplan-Meir estimates.

Results:

The cohort included 93 patients [52% (<65grp); 48% (≥65grp)]. The mean age at diagnosis was 59 ± 20 years (range 16-91). Specifically, the age of the <65grp was 43 ± 14 compared to 76 ± 7 for ≥ 65grp. Median follow-up was 4.6 years in the<65grp and 2.0 years in the 65grp. There were no significant differences in gender, race, BMI, or smoking status. At diagnosis, ≥65grp had 3 times as many comorbidities as <65grp (p <0.0001). The ≥65grp also had more renal involvement (73 vs 50%; p=0.02). The <65grp had more sinus (79 vs 44%; p=0.001) and skin (38 vs 18%; p=0.03) involvement. There were no differences in lung, ocular, neurologic, cardiovascular, gastrointestinal, musculoskeletal, constitutional, or hematologic/DVT findings. The elderly were also more likely to be MPO+ (69% MPO vs 31% PR3) whereas the younger were mostly PR3+ (17% MPO vs 83% PR3) (p <0.001).

Twenty percent of ≥65grp was hemodialyzed initially compared to 2 percent of <65grp (p=0.01). There was no difference in use of plasmapheresis, pulse steroids, cyclophosphamide (CYC) or rituximab (RTX). However, over the course of their illness, 21% of <65grp received both CYC and RTX vs none of the ≥65grp (p<0.001).

Although there was no significant difference between BVAS at presentation or VDI, the FFS at presentation was 2 times greater in the ≥65grp vs <65grp group (p<.0001). Interestingly, although both the mortality and FFS were increased in the ≥65grp, when this data was adjusted for age, the FFS did not have a significant association with the hazard of death (p=0.67) in the elderly. Although relapse was higher in the <65grp (56 vs 27%; p =0.0115), mortality was greater in the ≥65grp (98 vs 63%, p<0.001). There were no differences in complications.

Conclusion:

Our data indicate that clinical manifestations of AAV differ with age at presentation. There is also a striking age-related difference in distribution of ANCA subtype. While relapse was higher in younger patients, mortality was greater in the ≥65grp, possibly due to higher co-morbidity at presentation. A better understanding of the characteristic pattern of presentation between younger and older patients with AAV may have implications for appropriate management and help improve outcomes in this cohort of patients.

« Back to 2017 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/anca-associated-vasculitis-aav-in-younger-vs-older-patients-comparison-of-clinical-serologic-and-outcome-differences-and-their-implications-for-management/