Comparison of Patient Self-reported Data to Physician-driven Cohorts in Patients with Eosinophilic Granulomatosis with Polyangiitis

Irena Doubelt¹, Jason Springer ², Tanaz Kermani ³, Antoine Sreih ⁴, Cristina Burroughs ⁵, David Cuthbertson ⁵, Simon Carette ¹, Nader A. Khalidi ⁶, Curry L. Koening ⁷, Carol Langford ⁸, Carol A. McAlear ⁹, Larry W. Moreland ¹⁰, Paul Monach ¹¹, Dianne Shaw ¹², Philip Seo ¹³, Ulrich Specks ¹⁴, Kalen Young ¹⁵, Steven Ytterberg ¹⁴, Peter A. Merkel ¹⁶, Christian Pagnoux ¹ and VCRC Vasculitis Clinical Research Consortium ¹⁷, ¹Mount Sinai Hospital and University Health Network, Toronto, ON, Canada, ²Kansas University Medical Center, Kansas, MO, ³University of California Los Angeles, Los Angeles, CA, ⁴University of Pennsylvania, Philadelphia, PA, ⁵University of South Florida, Tampa, FL, ⁶McMaster University, Hamilton, ON, Canada, ⁷University of Utah Hospital, Salt Lake City, UT, ⁸Cleveland Clinic, Cleveland, OH, ⁹University of Pennsylvania - VCRC Project Manager, Philadelphia, PA, ¹⁰University of Pittsburgh, Pittsburgh, PA, ¹¹Brigham and Women's Hospital, Boston, MA, ¹²Vasculitis Foundation, North Carolina, ¹³Johns Hopkins Medicine, Baltimore, MD, ¹⁴Mayo Clinic College of Medicine, Rochester, MN, ¹⁵Vasculitis Foundation, Kansas City, MO, ¹⁶Univeristy of Pennsylvania, Philadelphia, PA, ¹⁷University of Pennsylvania, Division of Rheumatology, philadelphia

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: Churg-Strauss syndrome, online patient engagement, patient questionnaires and vasculitis, patient-reported outcome measures

Session Information

Date: Tuesday, November 12, 2019

Title: Vasculitis – ANCA-Associated Poster I

Session Type: Poster Session (Tuesday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Aligning perspectives of patients and physicians in the diagnosis and management of diseases is imperative, particularly in rare, chronic diseases such as vasculitis. We sought to compare patient-reported vs. physician-reported clinical manifestations, treatments, and outcomes in patients with eosinophilic granulomatosis with polyangiitis (EGPA).

Methods: Retrospective, comparative analysis of patients ≥18 years old with EGPA from Canada or the United States, from two separate databases: 1) Vasculitis Patient-Powered Research Network (VPPRN), a self-enrolled, secure patient portal with patient-entered data updated quarterly from 2013-2019, and 2) Vasculitis Clinical Research Consortium (VCRC) Longitudinal (LS) or the One-Time DNA (OT) studies, a combined physician-entered database (2003-2019). A few patients are in both databases, which are not presently linked due to protected health information policies. For validation of the diagnosis, patients in the VPPRN were excluded if they indicated that the diagnosis was not made by doctor and/or if they never used systemic glucocorticoids. Patients in the VCRC fulfilled the modified American College of Rheumatology 1990 criteria. Studied parameters included demographics, clinical manifestations, ANCA status, treatments received since diagnosis, and relapses.

Results: 208 patients self-registered with a diagnosis of EGPA in the VPPRN (195 had a validated diagnosis) and 354 patients were enrolled in the VCRC. 135 (69.2%) of the 195 VPPRN patients were female; 176 (92.1%) were white, 4 (2%) Asian, and 1 (0.5%) Black or African American. Mean age at first disease symptoms and diagnosis were 41.9 (SD ±14.9) years and 47.3 (±14.3) years, respectively. Method of diagnosis reported by patients was based on symptoms (91.3%), laboratory testing (80.5%), biopsy results (50.3%), and imaging (50.3%). At enrollment, 80 (41%) reported that the EGPA was active. Table 1 shows the main characteristics and outcomes of the VPPRN patients compared to those from the VCRC. Patients in the VPPRN were predominantly female, slightly younger at diagnosis than in the VCRC cohort, and reported more frequent disease manifestations in almost all organ systems, but less lung disease, and similar frequencies of asthma, cardiac and gastrointestinal manifestations. With a mean follow-up of 2.2 (±1.1) years post-enrollment, 63 (32.3%) of the VPPRN patients reported at least 1 relapse post-enrollment, which was comparable to the VCRC patients.

Conclusion: This analysis comparing patient vs. physician data in EGPA showed some differences with patients generally reporting more manifestations of disease. These differences suggest that patient self-reported data can provide additional useful information. How to best combine patient-reported and physician-reported data for research studies in vasculitis warrants additional study.

tab1

Main characteristics of patients with eosinophilic granulomatosis with polyangiitis from the Vasculitis Patient-Powered Research Network and the Vasculitis Clinical Research Consortium

Disclosure: I. Doubelt, None; J. Springer, InflaRx, 9; T. Kermani, None; A. Sreih, Bristol-Meyers Squibb, 3, Bristol-Myers Squibb, 3; C. Burroughs, None; D. Cuthbertson, None; S. Carette, None; N. Khalidi, None; C. Koening, None; C. Langford, Bristol-Myers Squibb, 2, GlaxoSmithKline,, 2, ChemoCentryx, 2, Genentech, 2, Bristol-Myers Squibb, 5, 9, Abbvie, 9, AstraZeneca, 9; C. McAlear, None; L. Moreland, None; P. Monach, None; D. Shaw, None; P. Seo, None; U. Specks, None; K. Young, None; S. Ytterberg, None; P. Merkel, AbbVie, 5, AstraZeneca, 2, 5, Biogen, 5, Boeringher-Ingelheim, 2, 5, Bristol-Myers Squibb, 2, 5, Celgene, 2, 5, ChemoCentryx, 2, 5, CSL Behring, 5, Genentech/Roche, 2, 5, Genzyme/Sanofi, 5, GlaxoSmithKline, 2, 5, InflaRx, 5, Insmed, 5, Jannsen, 5, Kiniksa, 5, Kypha, 2, TerumoBCT, 2, UpToDate, 7; C. Pagnoux, ChemoCentryx, 5, Chemocentryx, 5, Genetech/Roche, 5, Genzyme/Sanofi, 5, GlaxoSmithKline, 5, Hoffman-La Roche, 2, 5, 8, Hoffman-LaRoche, 2, 5, 8, Sanofi, 5; V. Vasculitis Clinical Research Consortium, None.

To cite this abstract in AMA style:

Doubelt I, Springer J, Kermani T, Sreih A, Burroughs C, Cuthbertson D, Carette S, Khalidi N, Koening C, Langford C, McAlear C, Moreland L, Monach P, Shaw D, Seo P, Specks U, Young K, Ytterberg S, Merkel P, Pagnoux C, Vasculitis Clinical Research Consortium V. Comparison of Patient Self-reported Data to Physician-driven Cohorts in Patients with Eosinophilic Granulomatosis with Polyangiitis [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/comparison-of-patient-self-reported-data-to-physician-driven-cohorts-in-patients-with-eosinophilic-granulomatosis-with-polyangiitis/. Accessed .

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