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Background: AAV (ANCA-associated vasculitis) is a group of progressive, rare, severe autoimmune diseases 1,2 . AAV can affect blood vessels in different parts of the body resulting in damage to vital organs such as the lungs, kidneys, nervous system, gastrointestinal system, skin, eyes, and heart. 2 There are currently no approved therapies for remission-induction in patients with AAV. The current treatment armamentarium for AAV is comprised of various immunosuppressive therapies in combination with steroid treatment. Understanding clinical practice gaps in the management of AAV, can inform development of tools to improve physician practices.
Objectives: This medical education activity aims to assess physicians’ knowledge on the various manifestations of antineutrophil cytoplasmic antibody-associated vasculitis (AAV), current guideline-recommended treatment strategies for remission induction in patients with AAV, as well as recent clinical trial data for combination therapies used for remission induction.
Methods: A 24-question survey consisting of multiple-choice knowledge and case-based questions was made available to nephrologists and rheumatologists without monetary compensation or charge. The questions were designed to evaluate knowledge regarding the various manifestations of AAV and the results from clinical trials that have compared the efficacy of combination therapies used for remission induction in patients with AAV. As well as application of guideline-recommended therapies and clinical trial data for remission induction in patients with AAV within clinical practice. The survey launched online on a website dedicated to continuous professional development. (
Results: 363 nephrologists and 190 rheumatologists completed the survey. Physicians demonstrated gaps in the following areas:
| Topic | Incorrect Responses to Knowledge and Clinical Decision-Making Questions (% ) | |
| Nephrologists | Rheumatologists | |
| Systemic diseases associated with AAV | 59% | 45% |
| How to confirm diagnosis of AAV | 42% | 25% |
| Therapy selection to induce remission that would be consistent with guidelines recommendations | 71% | 51% |
| Guideline-recommended therapy for patients that do not respond to the induction regimen | 32% | 34% |
| Definition of refractory disease | 95% | 94% |
| Most effective maintenance strategy for a patient once remission is achieved | 80% | 64% |
| Where would an emerging therapy such as a C5a receptor inhibitor fit into the therapeutic armamentarium of AAV? | 62% | 47% |
| What are the guideline-recommended therapies to reduce remission in patients without organ-threatening disease? | 71% | 51% |
| Most effective maintenance strategy for a patient once remission is achieved | 80% | 64% |
| Guideline-recommendations on length of time to continue maintenance therapy | 31% | 35% |
Conclusion: This educational research on assessment of physicians’ (nephrologists and rheumatologists) clinical practices yielded important insights into clinical gaps related to understanding of the disease pathophysiology and progression of AAV, guideline recommendations on diagnosing and managing AAV with guideline-directed medical therapies (GDMTs), strategies for the management of relapsing and refractory disease in AAV and positioning of emerging therapies in the treatment paradigm.
REFERENCES:
[1]www.medscape.org/viewarticle/920320.
[2]Hutton HL, et al. Semin Nephrol 2017;37(5):418–35.
[3]Al-Hussain T, et al. Adv Anat Pathol 2017;24(4):226–34.
Disclosure of Interests: Sarah Mendly Grant/research support from: Supported by an independent educational grant from Vifor Pharma, George Boutsalis Grant/research support from: Supported by an independent educational grant from Vifor Pharma