• Masayoshi Harigai, Kenji Nagasaka, Koichi Amano, Masashi Bando, Hiroaki Dobashi, Tamihiro Kawakami, Kan Kishibe, Yohko Murakawa, Joichi Usui, Takashi Wada, Eiichi Tanaka, Eishu Nango, Takeo Nakayama, Michi Tsutsumino, Kunihiro Yamagata, Sakae Homma, and Yoshihiro Arimua.
• a Division of Epidemiology and Pharmacoepidemiology, Department of Rheumatology, School of Medicine , Tokyo Women's Medical University , Tokyo , Japan.
• Mod Rheumatol. 2019 Jan 1; 29 (1): 20-30.

ObjectiveThe 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.MethodsThe 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.ResultsFor 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.ConclusionThe revised CPG has demonstrated evidence-based treatment recommendations for AAV.

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