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Mod Rheumatol. 2019 Jan;29(1):20-30. doi: 10.1080/14397595.2018.1500437. Epub 2018 Sep 10.

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.

Author information

1
a Division of Epidemiology and Pharmacoepidemiology, Department of Rheumatology, School of Medicine , Tokyo Women's Medical University , Tokyo , Japan.
2
b Department of Rheumatology , Ome Municipal General Hospital , Tokyo , Japan.
3
c Department of Pharmacovigilance, Graduate School of Medical and Dental Sciences , Tokyo Medical and Dental University , Tokyo , Japan.
4
d Department of Rheumatology and Clinical Immunology, Saitama Medical Center , Saitama Medical School , Saitama , Japan.
5
e Division of Pulmonary Medicine, Department of Medicine , Jichi Medical University , Tochigi , Japan.
6
f Division of Hematology , Rheumatology and Respiratory Medicine Department of Internal Medicine Faculty of Medicine Kagawa University , Kagawa , Japan.
7
g Department of Dermatology , St. Marianna University School of Medicine , Kanagawa , Japan.
8
h Department of Otolaryngology-Head and Neck Surgery , Asahikawa Medical University , Hokkaido , Japan.
9
i Department of Rheumatology , Shimane University Faculty of Medicine , Shimane , Japan.
10
j Department of Nephrology, Faculty of Medicine , University of Tsukuba , Ibaraki , Japan.
11
k Department of Nephrology and Laboratory Medicine, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences , Kanazawa University , Ishikawa , Japan.
12
l Department of Rheumatology, School of Medicine , Tokyo Women's Medical University , Tokyo , Japan.
13
m Department of General Medicine , Tokyo Kita Medical Center , Tokyo , Japan.
14
n Department of Health Informatics, Graduate School of Medicine and Public Health , Kyoto University , Kyoto , Japan.
15
o Department of Respiratory Medicine , Toho University Omori Medical Center , Tokyo , Japan.
16
p Division of Nephrology and Rheumatology, First Department of Internal Medicine , Kyorin University School of Medicine , Tokyo , Japan.

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; GRADE system; clinical practice guideline; granulomatosis with polyangiitis; microscopic polyangiitis

PMID:
30001655
DOI:
10.1080/14397595.2018.1500437
[Indexed for MEDLINE]

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