American College of Rheumatology (ACR) Vasculitis ...

1 American College of Rheumatology (ACR). Vasculitis Guideline ANCA-Associated Vasculitis Project Plan July 2018. PARTICIPANTS. Core Oversight Team John Stone, MD, MPH. Sharon Chung, MD, MAS (Principal Sangeeta Sule, MD, PhD. Investigator/Voting Panel Leader) Robert Sundel, MD, BA. Gary Hoffman, MD (Content Expert) Ann Warner, MD. Carol Langford, MD (Content Expert). Mehrdad Maz, MD (Content Expert) Expert Panel Antoine Sreih, MD (Content Expert) Ora Gewurz-Singer, MD. Reem Mustafa, , PhD, MPH (Literature Rula Hajj-Ali, MD. Review Leader) Eric Matteson, MD, MPH. Gordon Guyatt, MD (GRADE Expert) Robert Spiera, MD. Linda Wagner-Weiner, MD. Literature Review Team Ken Warrington, MD. Kevin Byram, MD. Anisha Dua, MD, MPH Patient Panel Nedaa Husainat, MD TBD. Karen E. James, MD, Mohamad Kalot, MD ACR Staff Yih Chang Chen Lin, MD Robin Lane Catherine Najem, MD Amy S.

2 Turner Amit Shah, MD, MPH Regina Parker Jason Springer, MD, MS. Marat Turgunbaev, MD, MPH. Alexandra Villa-Forte, MD, MPH. Voting Panel Amy Archer, MD, PhD. Doyt Conn, MD. Peter C. Grayson, MD, Maria Ibarra, MD. Susan Kim, MD. Peter Merkel, MD, MPH. Rennie Rhee, MD. Phil Seo, MD, MHS. 1. American College of Rheumatology (ACR). Vasculitis Guideline ANCA-Associated Vasculitis Project Plan July 2018. 1. 2 ORGANIZATIONAL LEADERSHIP AND SUPPORT. 3. 4 This project of the American College of Rheumatology (ACR) has the broad objective of developing an 5 evidence-based clinical practice guideline related to the treatment and management of systemic 6 Vasculitis . 7. 8 BACKGROUND. 9. 10 The ACR has previously not developed guidelines for the management of systemic Vasculitis . The 11 diagnosis, treatment, and monitoring of these diseases can be challenging given their rarity and the 12 paucity of large, randomized clinical trials to guide therapy for some vasculitic diseases.

3 Therefore, the 13 ACR convened the Vasculitis Guideline core leadership team to develop evidence-based guidelines for 14 the management of systemic Vasculitis . The group was encouraged to scope broadly, without mandate 15 to cover a specific type of Vasculitis . It was recognized that one set of guidelines could not cover the 16 entire spectrum of vasculitic diseases, and that vasculitides not addressed in this initial effort could be 17 covered in future guidelines. 18 At the group's first in-person meeting in June 2017, the Core Oversight Team, Voting Panel, and Expert 19 Panel discussed the scope that should be covered in this initial guideline effort. The 2012 Revised 20 International Chapel Hill Consensus Conference Nomenclature (1) was used as the basis for categorizing 21 the vasculitides to be considered.

4 For this initial effort, the vasculitides in the major categories large, 22 medium, and small vessel Vasculitis were prioritized given their prevalence compared to other 23 categories of Vasculitis . After discussion, the group members elected to first focus on large and medium 24 vessel vasculitides, due to the scope of the project, need for clinical guidelines for these diseases and 25 the available evidence upon which these guidelines could be based. The project plan presenting the 26 committee's work on large and medium vessel Vasculitis was presented to the Rheumatology community 27 in March 2018. After development of that project plan, the committee began the current effort to 28 develop guidelines for small vessel Vasculitis . At this time, guidelines are being developed for anti- 29 neutrophil cytoplasmic antibody (ANCA)-associated Vasculitis due to the relative prevalence and 30 availability of literature and therapeutic options.

5 We recognize that management guidelines for other 31 types of small vessel Vasculitis such as IgA Vasculitis , cryoglobulinemic Vasculitis , hypocomplementic 32 urticarial Vasculitis , and anti-glomerular basement membrane disease are also needed. Development of 33 guidelines for these vasculitides may be addressed at a later time. 34 Using the Chapel Hill Consensus Conference nomenclature, the ANCA-associated vasculitides included in 35 this effort are granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic 2. American College of Rheumatology (ACR). Vasculitis Guideline ANCA-Associated Vasculitis Project Plan July 2018. 36 granulomatosis with polyangiitis (EGPA). Renal limited ANCA-associated Vasculitis is not being addressed 37 specifically in this effort, but studies of renal limited ANCA-associated Vasculitis will be included in the 38 literature review.

6 Many questions presented in this project plan consider GPA and MPA together. We 39 recognize that these are different diseases for which disease specific management approaches exist, 40 particularly for GPA. However, we have grouped these two diseases together for many treatment 41 questions since pivotal trials have enrolled and presented results for these diseases together, and data 42 may be limited regarding the treatment outcomes for these diseases individually. The Vasculitis 43 Guideline group intends for these guidelines to be applicable to both adults and children affected by 44 these diseases. Thus, the group is comprised of both adult and pediatric rheumatologists, and the 45 questions addressed in this guideline apply to both adults and children. 46. 47 OBJECTIVES. 48. 49 The objective of this project is to develop recommendations informing the use of laboratory testing, 50 pharmacologic treatments, and non-pharmacologic interventions for the management of ANCA- 51 associated Vasculitis : granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and 52 eosinophilic granulomatosis with polyangiitis (EGPA).

7 53. 54 Specifically, we aim to: 55. 56 1. Develop recommendations for the use of laboratory and imaging studies that inform the 57 management of ANCA-associated Vasculitis . 58 2. Develop recommendations for the use of glucocorticoids, non-glucocorticoid and biologic 59 immunosuppressive agents, and non-pharmacologic interventions for the management of 60 ANCA-associated Vasculitis based on considerations of both efficacy and safety. 61. 62 METHODS. 63. 64 Identification of Studies 65. 66 Literature search strategies, based on PICO questions (Population/patients, Intervention, Comparator, 67 and Outcomes; see Appendix A) will be developed by the principal investigators, systematic literature 68 review leader, and a research librarian, with input from the Core Team. The search strategies will be 69 peer reviewed by another medical librarian using Peer Review of Electronic Search Strategies (PRESS).

8 70 (2). Searches will be performed in OVID Medline (1946 +), Embase (1974 +), the Cochrane Library, and 71 PubMed (mid-1960s +). 3. American College of Rheumatology (ACR). Vasculitis Guideline ANCA-Associated Vasculitis Project Plan July 2018. 72. 73 The search strategies will be developed using the controlled vocabulary or thesauri language for each 74 database: Medical Subject Headings (MeSH) for OVID Medline, PubMed and Cochrane Library; and 75 Emtree terms for Embase. Text words will also be used in OVID Medline, PubMed, and Embase, and 76 keyword/title/abstract words in the Cochrane Library. 77. 78 Search Limits 79. 80 Only English language articles will be retrieved. 81. 82 Grey Literature 83. 84 The websites of appropriate agencies, such as the Agency for Healthcare Research and Quality (AHRQ), 85 will be searched for peer-reviewed reports not indexed by electronic databases.

9 86. 87 Literature Search Update 88. 89 Literature searches will be updated just before the voting panel meeting to ensure completeness. 90. 91 Inclusion/Exclusion Criteria 92. 93 See PICO questions (Appendix A), which outline the defined patient population, interventions, 94 comparators and outcomes. 95. 96 Management of Studies and Data 97. 98 References and abstracts will be imported into bibliographic management software (Reference 99 Manager) (3), duplicates removed, and exported to Distiller SR, a web-based systematic review manager 100 (4). Screening and data abstraction forms will be created in Distiller SR. Search results will be divided 101 among reviewers, and two reviewers will screen each title/abstract, with disagreements at the 102 title/abstract screening stage defaulting to inclusion for full manuscript review.

10 Following the same dual 103 review process, disagreements at the full manuscript screening stage will be discussed and adjudicated 104 by the literature review leadership, if necessary. 105. 106. 107. 108. 4. American College of Rheumatology (ACR). Vasculitis Guideline ANCA-Associated Vasculitis Project Plan July 2018. 109 Phases 110. 111 1. A search for randomized controlled trials and observational studies about interventions aimed 112 at the pharmacologic treatments and non-pharmacologic interventions for the management of 113 ANCA-associated Vasculitis (GPA, MPA, and EGPA) will be performed to determine existing 114 studies covering outcomes of interest. Studies of renal limited ANCA-associated Vasculitis will 115 not be excluded. Subsequently, identified studies will be assessed using the RevMan (5) and 116 GRADE Pro tools (6).