Background/Purpose: To provide a feasible and sustainable platform for conducting patient-centered research in vasculitis, a prospective, international, internet-based registry of patients with vasculitis has been established. The aim of this study is to describe the self-reported clinical features of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) from this registry.

Methods: Patients (or caregivers) with different forms of vasculitis can join the web-based registry. All information is self-reported through standardized forms, including type of vasculitis, symptoms at disease onset and later in the disease course, and information regarding how diagnosis was made. For each manifestation, patients can respond with “Yes”, “No”, or “I don’t know”. Only patients with GPA or MPA were included in this analysis. Other analyses demonstrated that in this cohort the accuracy of the self-reported diagnosis of GPA or MPA is > 90%.

Results: The study cohort included 762 patients (68% female) with GPA and 164 patients (81% female) with MPA.

GPA Cohort: 98% of patients reported a physician-confirmed diagnosis. Commonly reported means of diagnosis were combinations of laboratory testing (74%), symptoms (70%), biopsy (62%), and imaging (38%). 562 patients (74%) reported having at least one biopsy, including skin (70 patients), kidney (283 patients), lung (192 patients), nasal/sinus (146 patients), nerve (10 patients), other (36 patients). ANCA was reported in 652/692 (94%) patients who knew their ANCA status. Clinical manifestations at entry into the cohort are summarized in Table 1.

MPA Cohort: 99% of patients reported a physician-confirmed diagnosis. Commonly reported means of diagnosis were combinations of laboratory testing (79%), symptoms (67%), biopsy (77%), and imaging (42%). 131 patients (80%) reported having at least one biopsy, including kidney (103 patients), lung (20 patients), skin (15 patients), nerve (6 patients), and nasal (4 patients). ANCA was reported as positive in 147/153 (96%) patients who knew their ANCA status. Clinical manifestations at entry into the cohort are summarized in Table 1.

Conclusion: In this large cohort patients with a self-reported diagnosis of GPA or MPA, disease manifestations were consistent with each type of ANCA-associated vasculitis. As expected, sinus manifestations were more frequently reported in GPA cohort while renal manifestations were more frequent in the MPA cohort. Given the rarity of these and other vasculitides, conducting some types of research through online registries may provide an efficient alternative to in-person, center-of-excellence clinical trials.

Table 1_VPPRN_pt_characteristics

« Back to 2019 ACR/ARP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-characteristics-of-a-cohort-of-patients-with-a-self-reported-diagnosis-of-granulomatosis-with-polyangiitis-or-microscopic-polyangiitis/