2017 Clinical practice guidelines of the Japan Research Committee of the Ministry of Health, Labour, and Welfare for Intractable Vasculitis for the management of ANCA-associated vasculitis

Abstract

Objective: The Japan Research Committee for Intractable Vasculitis has fully revised the clinical practice guidelines (CPG) for the management of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) to improve and standardize the medical treatment of the disease in Japan.

Methods: The previous CPG was published in a classical review style in Japanese in 2011 and 2014. We adopted the Grading of Recommendations Assessment, Development and Evaluation system for this revision, and various stakeholders, including patients, participated in it. The expected users of this CPG are AAV patients in Japan and their families and healthcare professionals, including both AAV specialists and non-specialists. We set clinical questions concerning the three important clinical topics of remission induction therapy, plasma exchange, remission maintenance therapy, and developed eight recommendation statements.

Results: For remission induction therapy for newly developed AAV, we weakly recommend glucocorticoid (GC) plus intravenous cyclophosphamide pulse (IVCY) or oral cyclophosphamide (POCY) rather than GC alone, and IVCY rather than POCY. We also weakly recommend CY rather than rituximab. In the case of AAV with severe renal impairment, we weakly recommend plasma exchange as a conjunction therapy. We weakly recommend azathioprine for remission maintenance therapy.

Conclusion: The revised CPG has demonstrated evidence-based treatment recommendations for AAV.

Keywords:

• ANCA-associated vasculitis
• clinical practice guideline
• GRADE system
• microscopic polyangiitis
• granulomatosis with polyangiitis

Acknowledgements

JPVAS would like to thank Morio Aihara (Aihara Clinic, Hirosaki University), Erika Ota (St. Luke's International Hospital), Shoko Kasai (Tokyo Medical and Dental University), and Taku Yabuki (Tochigi medical centre) for their external evaluation. JPVAS sincerely appreciate Tomoko Morimasa and Naoko Suwabe from the Japan Library Association who performed the literature search for SR. JPVAS is grateful to the Japanese Respiratory Society, Japanese Society of Nephrology, and Japan College of Rheumatology for inviting public comments.

Conflict of interest

To maintain strictness and transparency when developing CPG, managing the conflicts of interest (COI) of all healthcare professionals is necessary. The organizing committee discussed a financial COI disclosure form and its management, and decided to request the Japan College of Rheumatology to manage this process. Members of the organizing committee, CPG panel, SR team, and secretariat submitted the COI disclosure form to the Japan College of Rheumatology. To minimize academic COI, the organizing committee assembled physicians with diverse specialties to be members of the CPG panel and SR team, and made their best effort to exclude the preferences of each member and their scientific organization, and the possible effects of competition among healthcare professionals and/or organizations. The academic COI of the organizing committee, the CPG panel, and SR team are described in the Supplementary Table.

MH received research grants and lecture fee from Teijin Pharma Ltd., Chugai Pharmaceutical Co., Ltd. and Mitsubishi Tanabe Pharma Corporation and served as an adviser for Kissei Pharmaceutical Co., Ltd. and Zenyaku Kogyo. KA received research grants from Chugai Pharmaceutical Co., Ltd. and speaking fee from Mitsubishi Tanabe Pharma Corporation, Eli Lilly Japan K.K., and Pfizer Japan Inc. YM received research grants from Chugai Pharmaceutical Co., Ltd., Teijin Pharma Ltd., and Mitsubishi Tanabe Pharma Corporation, served as paid instructor for Kissei Pharmaceutical Co., Ltd., served as speaker’s bureau for Mitsubishi Tanabe Pharma Corporation, Chugai Pharmaceutical Co., Ltd., Takeda Pharmaceutical Company Limited, and Teijin Pharma Ltd.

Additional information

Funding

Research grants to JPVAS from the Ministry of Health, Labour, and Welfare of Japan (H26-nanchi-nanchitou (nan)-ippan-044) and the Department of Pharmacovigilance, Tokyo Medical and Dental University were used to pay for venues, traveling and correspondence expenses, and meals. No compensation was made to the members involved in the development of the CPG.