2492: The Impact of Neurologic Involvement in ANCA-Associated Vasculitis on Self-Reported Health-Related Quality of Life

Rula Hajj-Ali¹, Chao Zhang², Renee Borchin³, Jenn Gordon⁴, David Cuthbertson³, Carol McAlear⁵, Christine Yeung⁵, David Badenoch⁶, Cristina Burroughs³ and Peter Merkel⁵, ¹Cleveland Clinic, Cleveland, OH, ²Cleveland Clinic Foundation, Cleveland, OH, ³University of South Florida, Tampa, FL, ⁴Vasculitis Patient Powered Research Network (VPPRN), Philadelphia, PA, ⁵University of Pennsylvania, Philadelphia, PA, ⁶Vasculitis Patient Powered Research Network (VPPRN), Los Angeles, CA

Background/Purpose: Neurologic involvement (NI) is a common manifestation in patients with ANCA-associated vasculitis (AAV) and can lead to chronic pain and disability. This project assessed the impact of NI on health-related quality of life (HRQoL) in AAV.

Methods: Retrospective analysis of data provided from 2014-2022 by patients ≥18 years old with AAV [eosinophilic granulomatosis with polyangiitis (EGPA), granulomatosis with polyangiitis (GPA), or microscopic polyangiitis (MPA)] enrolled in the Vasculitis Patient-Powered Research Network (VPPRN), an online registry. The VPPRN collects data regarding type of vasculitis, disease manifestations, information on how and when the diagnosis of vasculitis was established, and measures of HRQoL, including from multiple Patient-Reported Outcome Measurement Information System (PROMIS) domains. PROMIS measures generate T-scores with the mean T-score = 50 with a standard deviation of 10 in the reference population (U.S. general population). Data were analyzed according to NI involvement.

Results: Data from 1465 patients with AAV with available information on NI were included in this analysis; 901 of 1465 (61.5%) patients reported NI. Table 1 summarizes the demographic and clinical characteristics of patients with or without NI. Compared to patients without NI, those with NI were older at the time of diagnosis of AAV, were more likely to be diagnosed with GPA (vs. EGPA or MPA), be positive for ANCA, and more likely to have constitutional, musculoskeletal, skin, mucous membranes, ear/nose/throat, cardiovascular, pulmonary, and thrombotic manifestations of disease. The rate of renal involvement was the same among patients with or without NI. Patients with NI reported greater severity of vasculitis. There was no significant difference in the medications ever received among patients with NI vs patients with no NI.

Overall, patients with AAV reported reduced HRQoL in almost all domains compared to a reference population. Compared to patients without NI, patients with NI report higher scores for fatigue, depression, sleep disturbance, and pain interference, and lower scores for anxiety, social role, and physical function (Figure2).

Conclusion: NI, as reported directly by patients, is common in AAV, occurring in approximately two-thirds of patients. This rate is higher than has been reported through clinician-documented data, implying that physicians may be under-appreciating the extent of NI in AAV. NI is more common in GPA, compared to EGPA or MPA. Patients with NI have involvement of more organ systems than patients without NI. NI in patients with AAV has a negative impact on multiple domains of illness and health-related quality of life. These patient-reported data are informative for clinicians and patients with AAV and should raise the awareness of the impairment in patients with NI in AAV






R. Hajj-Ali: Amgen, 2, GlaxoSmithKlein(GSK), 2, uptodate, 9; C. Zhang: None; R. Borchin: None; J. Gordon: Excision Biotherapeutics, 3; D. Cuthbertson: None; C. McAlear: None; C. Yeung: None; D. Badenoch: None; C. Burroughs: None; P. Merkel: AbbVie/Abbott, 5, Amgen, 2, 5, ArGenx, 2, AstraZeneca, 2, 5, Boehringer-Ingelheim, 2, 5, Bristol-Myers Squibb(BMS), 2, 5, Cabaletta, 2, CSL Behring, 2, Eicos, 5, Electra, 5, Genentech, 5, GlaxoSmithKlein(GSK), 2, 5, HiBio, 2, InflaRx, 2, 5, Janssen, 2, Jubilant, 2, Kyverna, 2, 11, MiroBio, 2, Neutrolis, 5, Novartis, 2, NS Pharma, 2, Q32, 2, Regeneron, 2, Sanofi, 2, Sparrow, 2, Takeda, 2, 5, UpToDate, 9, Visterra, 2.