Reclassification of the overlap syndrome of Behçet’s disease and antineutrophil cytoplasmic antibody-associated vasculitis in patients with Behçet’s disease
Background/Aims This study applied the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with Behçet’s disease (BD) to investigate the proportion and cli...
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The Korean Association of Internal Medicine
2025-01-01
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| Series: | The Korean Journal of Internal Medicine |
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| Online Access: | http://kjim.org/upload/kjim-2024-011.pdf |
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| author | Tae Geom Lee Jang Woo Ha Jason Jungsik Song Yong-Beom Park Sang-Won Lee |
| author_facet | Tae Geom Lee Jang Woo Ha Jason Jungsik Song Yong-Beom Park Sang-Won Lee |
| author_sort | Tae Geom Lee |
| collection | DOAJ |
| description | Background/Aims This study applied the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with Behçet’s disease (BD) to investigate the proportion and clinical implications of the reclassification to the overlap syndrome of BD and AAV (OS-BD-AAV). Methods We included 280 BD patients presenting with ANCA positivity but without medical conditions mimicking AAV at diagnosis. Demographic data, items from the 2014 revised International Criteria for BD and 2022 American College of Rheumatology and European Alliance of Associations for Rheumatology criteria for AAV, ANCA positivity, and laboratory results were recorded as clinical data at diagnosis. A total score ≥ 5 indicated microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA), whereas a total score ≥ 6 indicated a diagnosis of eosinophilic GPA (EGPA). Results The overall reclassification rate of OS-BD-AAV was 8.6%. Of the 280 patients, 16 (5.7%) and 8 (2.9%) were reclassified as having OS-BD-MPA and OS-BD-GPA, respectively; none were classified as having OS-BD-EGPA. ANCA, myeloperoxidase-ANCA (P-ANCA), proteinase 3-ANCA (C-ANCA) positivity, hearing loss, and interstitial lung disease (ILD) at diagnosis were more common in patients with OS-BD-AAV than in those without. ANCA positivity and ILD at BD diagnosis contributed to the reclassification of OS-BD-AAV. However, hearing loss was not considered a major contributor to BD due to its possibility of developing as a manifestation of BD. Conclusions To our knowledge, this is the first study to demonstrate the reclassification rate (8.6%) of patients with BD and ANCA results at diagnosis as OS-BD-AAV. |
| format | Article |
| id | doaj-art-2732f0441a7946a09debb2f3c0d43fe6 |
| institution | OA Journals |
| issn | 1226-3303 2005-6648 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | The Korean Association of Internal Medicine |
| record_format | Article |
| series | The Korean Journal of Internal Medicine |
| spelling | doaj-art-2732f0441a7946a09debb2f3c0d43fe62025-08-20T01:55:09ZengThe Korean Association of Internal MedicineThe Korean Journal of Internal Medicine1226-33032005-66482025-01-0140113514710.3904/kjim.2024.011170989Reclassification of the overlap syndrome of Behçet’s disease and antineutrophil cytoplasmic antibody-associated vasculitis in patients with Behçet’s diseaseTae Geom Lee0Jang Woo Ha1Jason Jungsik Song2Yong-Beom Park3Sang-Won Lee4 Department of Medicine, Yonsei University College of Medicine, Seoul, Korea Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, KoreaBackground/Aims This study applied the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with Behçet’s disease (BD) to investigate the proportion and clinical implications of the reclassification to the overlap syndrome of BD and AAV (OS-BD-AAV). Methods We included 280 BD patients presenting with ANCA positivity but without medical conditions mimicking AAV at diagnosis. Demographic data, items from the 2014 revised International Criteria for BD and 2022 American College of Rheumatology and European Alliance of Associations for Rheumatology criteria for AAV, ANCA positivity, and laboratory results were recorded as clinical data at diagnosis. A total score ≥ 5 indicated microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA), whereas a total score ≥ 6 indicated a diagnosis of eosinophilic GPA (EGPA). Results The overall reclassification rate of OS-BD-AAV was 8.6%. Of the 280 patients, 16 (5.7%) and 8 (2.9%) were reclassified as having OS-BD-MPA and OS-BD-GPA, respectively; none were classified as having OS-BD-EGPA. ANCA, myeloperoxidase-ANCA (P-ANCA), proteinase 3-ANCA (C-ANCA) positivity, hearing loss, and interstitial lung disease (ILD) at diagnosis were more common in patients with OS-BD-AAV than in those without. ANCA positivity and ILD at BD diagnosis contributed to the reclassification of OS-BD-AAV. However, hearing loss was not considered a major contributor to BD due to its possibility of developing as a manifestation of BD. Conclusions To our knowledge, this is the first study to demonstrate the reclassification rate (8.6%) of patients with BD and ANCA results at diagnosis as OS-BD-AAV.http://kjim.org/upload/kjim-2024-011.pdfbehcet syndromeantibodies, antineutrophil cytoplasmicvasculitisclassification |
| spellingShingle | Tae Geom Lee Jang Woo Ha Jason Jungsik Song Yong-Beom Park Sang-Won Lee Reclassification of the overlap syndrome of Behçet’s disease and antineutrophil cytoplasmic antibody-associated vasculitis in patients with Behçet’s disease The Korean Journal of Internal Medicine behcet syndrome antibodies, antineutrophil cytoplasmic vasculitis classification |
| title | Reclassification of the overlap syndrome of Behçet’s disease and antineutrophil cytoplasmic antibody-associated vasculitis in patients with Behçet’s disease |
| title_full | Reclassification of the overlap syndrome of Behçet’s disease and antineutrophil cytoplasmic antibody-associated vasculitis in patients with Behçet’s disease |
| title_fullStr | Reclassification of the overlap syndrome of Behçet’s disease and antineutrophil cytoplasmic antibody-associated vasculitis in patients with Behçet’s disease |
| title_full_unstemmed | Reclassification of the overlap syndrome of Behçet’s disease and antineutrophil cytoplasmic antibody-associated vasculitis in patients with Behçet’s disease |
| title_short | Reclassification of the overlap syndrome of Behçet’s disease and antineutrophil cytoplasmic antibody-associated vasculitis in patients with Behçet’s disease |
| title_sort | reclassification of the overlap syndrome of behcet s disease and antineutrophil cytoplasmic antibody associated vasculitis in patients with behcet s disease |
| topic | behcet syndrome antibodies, antineutrophil cytoplasmic vasculitis classification |
| url | http://kjim.org/upload/kjim-2024-011.pdf |
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