Session Type: Poster Session (Tuesday)
Session Time: 9:00AM-11:00AM
Background/Purpose: To determine the reliability of self-reported diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) from a large, international, internet-based cohort.
Methods: The web-based registry includes data provided by patients (or caregivers) with different forms of primary vasculitis collected through standardized forms including type of vasculitis, disease manifestations, and information regarding how diagnosis was made. This analysis included 150 randomly-selected patients with GPA (out of 484) with complete data and all patients with MPA and EGPA. Primary outcome was percentage of patients who met the 1990 American College of Rheumatology (ACR) classification criteria and/or 2012 Chapel Hill Consensus Conference (CHCC) definitions for vasculitis. ACR criteria for GPA were modified to include ANCA positivity.
Results: The study cohort included 150 patients (70% female) with GPA, 164 patients (81% female) with MPA and 116 patients (66% female) with EGPA.
GPA: Clinical manifestations included sinus/nasal (84%), pulmonary (63%, alveolar hemorrhage in 34%) and renal involvement (57%). ANCA was reported as positive in 123 of 134 patients (92%) who knew their ANCA status. 70% patients reported having at least one biopsy and 61% patients reported diagnosis by a biopsy. Overall, 96% patients met either the modified ACR criteria or the CHCC definition. 93% patients met both the modified ACR criteria and CHCC definition.
MPA: Clinical manifestations included pulmonary (62%, alveolar hemorrhage in 36%), renal involvement (84%), and neurologic (65%). ANCA was reported as positive in 147 of 153 patients (96%) who knew their ANCA status. Biopsies were performed in 131 patients (80%) with 77% patients reporting diagnosis by biopsy. 95% patients met the CHCC definition.
EGPA: Clinical manifestations included asthma (95%), nasal/sinus (94%), peripheral nerve involvement (88%), non-asthma pulmonary disease (74%). ANCA was reported as positive in 33 of 79 patients (42%) who knew their ANCA status. Peripheral eosinophilia was reported in 108 of 112 patients (96%). 100 patients (86%) met ≥ 4 ACR criteria and 75 (65%) met the CHCC definition. All patients who met CHCC definitions also met ACR criteria.
Conclusion: Patient self-report of a diagnosis of ANCA-associated vasculitis is reliable with 86-96% of patients fulfilling the ACR criteria or CHCC definitions for their disease. Additional confirmation of the findings using medical records is currently underway. These results strongly support the validity of utilizing online, patient-generated data for clinical and epidemiological research in ANCA-associated vasculitis.
To cite this abstract in AMA style:Kermani T, Springer J, Sreih A, Shaw D, Young K, Burroughs C, Merkel P. Accuracy of Self-Reported Diagnosis of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/accuracy-of-self-reported-diagnosis-of-antineutrophil-cytoplasmic-antibody-associated-vasculitis/. Accessed July 11, 2020.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/accuracy-of-self-reported-diagnosis-of-antineutrophil-cytoplasmic-antibody-associated-vasculitis/