{"doi":"10.2332/allergolint.r-07-141","title":"ANCA-associated Vasculitis: Diagnostic Therapeutic Strategy","abstract":null,"journal":"Allergology International","year":2007,"id":654107,"datarank":0.7454719949364003,"base_score":4.969813299576001,"endowment":4.969813299576001,"self_citation_contribution":0.7454719949364003,"citation_network_contribution":0.0,"self_endowment_contribution":0.7454719949364003,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":143,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1707057,"name":"Shoichi Ozaki","orcid":null,"position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"ANCA-associated Vasculitis: Diagnostic Therapeutic Strategy","abstract":"Among small-vessel vasculitides, microscopic polyangiitis (MPA), Wegener's granulomatosis (WG), and allergic granulomatous angiitis (AGA) are known collectively as ANCA-associated vasculitis (AAV) because of the involvement of anti-neutrophil cytoplasmic antibodies (ANCA) as the common pathogenesis. Major target antigens of ANCA associated with vasculitis are myeloperoxidase (MPO) and proteinase 3 (PR3). MPO-ANCA is related to MPA and AGA, and PR3-ANCA is the marker antibody in WG. MPO-ANCA-associated vasculitis is more frequent in Japan, whereas PR3-ANCA-associated vasculitis is more common in Europe and USA. ANCA appears to induce vasculitis by directly activating neutrophils. Therefore, no immunoglobulins or complement components are detected in the vasculitis lesions; hence, AAV is called pauci-immune vasculitis (pauci = few/little). Untreated patients with severe AAV with multi-organ involvement have a poor prognosis, which is improved by combination therapy with cyclophosphamide and high-dose corticosteroid. Randomized controlled trials (RCT) regarding induction and maintenance of remission of AAV indicated that the rate of remission induction by the standard regimen is approximately 90% in 6 months, that maintenance of remission can be achieved with oral azathioprine as well as cyclophosphamide, and that methotrexate can be used only for non-renal mild AAV. As these data were obtained mostly in patients positive for PR3-ANCA, caution must be taken in applying these findings to Japanese patients, most of whom are positive for MPO-ANCA. A prospective study is now underway to clarify the effectiveness of the standard regimen in Japanese patients with MPO-ANCA-associated vasculitis. This article describes the diagnostic criteria and the recent evidence-based therapeutic strategy of AAV.","is_dataset_classified":null,"base_score":0.0,"endowment":0.0,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"17460438","pmcid":null,"openalex_id":null,"authors":[],"funders":[],"total_grants":0,"fwci":null,"citation_percentile":null,"influential_citations":0,"citation_trend":[],"oa_status":null,"license":"http://creativecommons.org/licenses/by-nc-nd/4.0/","oa_locations":[{"url":"http://api.elsevier.com/content/article/PII:S1323893015308522?httpAccept=text/xml","host_type":"publisher"},{"url":"http://api.elsevier.com/content/article/PII:S1323893015308522?httpAccept=text/plain","host_type":"publisher"},{"url":"https://doi.org/10.2332/allergolint.r-07-141","host_type":"Unpaywall"}],"fields_of_study":[],"mesh_terms":["Humans","Vasculitis","Churg-Strauss Syndrome","Cyclophosphamide","Methotrexate","Azathioprine","Adrenal Cortex Hormones","Peroxidase","Immunoglobulins, Intravenous","Immunosuppressive Agents","Antibodies, Antineutrophil Cytoplasmic","Biological Products","Immunologic Factors","Treatment Outcome","Drug Therapy, Combination","Remission Induction","Severity of Illness Index","Myeloblastin","Granulomatosis with Polyangiitis"],"keywords":[],"sdg_mappings":[],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-11T03:45:22.371487Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}