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Unclassifiable repeated antineutrophil cytoplasmic antibody (ANCA) positivity in diseases other than ANCA-associated vasculitis

 Hyeok Choi  ;  Yong-Beom Park  ;  Jaewoo Song  ;  Sang-Won Lee 
 ZEITSCHRIFT FUR RHEUMATOLOGIE, Vol.81(8) : 705-711, 2022-10 
Journal Title
Zeitschrift für Rheumatologie
Issue Date
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / diagnosis ; Antibodies, Antineutrophil Cytoplasmic ; Humans ; Kidney ; Renal Insufficiency, Chronic* ; Retrospective Studies
Antineutrophil cytoplasmic antibody ; Chronic kidney disease ; Interstitial lung disease ; Positivity ; Vasculitis
Background and objective: Antineutrophil cytoplasmic antibody (ANCA) is a specific autoantibody for ANCA-associated vasculitis (AAV). However, ANCA can be detected in various diseases other than AAV. Hence, in this study, we investigated and provided the name of diseases with repeated ANCA positivity and the frequency of each disease other than AAV.

Methods: We retrospectively screened the results of the tests of ANCA in 26,499 patients using the Clinical Data Repository System and included in this study only 173 patients with repeated ANCA positivity more than twice. 'Unclassifiable ANCA' was defined when ANCA was detected in patients with diseases other than AAV. 'Unclassifiable repeated ANCA' was also defined when unclassifiable ANCA was successively detected more than twice.

Results: Among rheumatic and autoimmune diseases, the most common disease with unclassifiable repeated ANCA was vasculitis undetermined (21.0%). In terms of cardiovascular and cerebrovascular diseases, the most common disease with unclassifiable repeated ANCA was atherosclerotic heart disease (12.1%). In terms of disorders in liver, kidneys and lungs, the most common disease with unclassifiable repeated ANCA was chronic kidney disease (51 cases, 29.5%). In addition, among infections with confirmed infectious pathogens, the most common pathogen with unclassifiable repeated ANCA was varicella-zoster virus (6.9%) followed by Candida (4.6%).

Conclusion: Overall, regardless of category, the common diseases with unclassifiable repeated ANCA were chronic kidney disease followed by interstitial lung disease and vasculitis undetermined. Thus, we carefully suggest that physicians should pay more attention to the development of AAV or vasculitis other than AAV and, furthermore, kidneys and lungs should be monitored regularly and closely in patients with unclassifiable repeated ANCA.
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1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Laboratory Medicine (진단검사의학교실) > 1. Journal Papers
Yonsei Authors
Park, Yong Beom(박용범)
Song, Jae Woo(송재우) ORCID logo https://orcid.org/0000-0002-1877-5731
Lee, Sang-Won(이상원) ORCID logo https://orcid.org/0000-0002-8038-3341
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