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Published on: February 8, 2019
Antineutrophil Cytoplasmic Antibody Positivity Is Associated with Vascular Involvement in Behçet's Disease
Minyoung Kevin Kim1, Hyeok Chan Kwon2, Jason Jungsik Song2,3
1Department of Medicine, Yonsei University College of Medicine, Seoul, Korea.
Insights
Antineutrophil cytoplasmic antibody (ANCA) positivity in Behçet's disease (BD) is linked to vascular issues at diagnosis, particularly in upper extremities and visceral arteries. However, ANCA positivity does not predict poor long-term outcomes in BD patients.
Area of Science:
- Rheumatology
- Immunology
- Vascular Medicine
Background:
- Behçet's disease (BD) is a multisystem inflammatory disorder.
- Vascular involvement is a major cause of morbidity and mortality in BD.
- The role of antineutrophil cytoplasmic antibodies (ANCA) in BD pathogenesis and prognosis is not fully understood.
Purpose of the Study:
- To investigate the association between ANCA positivity and vascular manifestations at diagnosis in BD patients.
- To determine if ANCA positivity predicts poor outcomes during follow-up in BD.
Main Methods:
- Retrospective review of medical records of 1060 BD patients.
- Diagnosis confirmed using the 2014 ICBD criteria (n=808) and 1990 ISG criteria (n=588).
- Evaluation of vascular involvement at diagnosis and adverse outcomes during follow-up.
Main Results:
- ANCA positivity was found in 2.2% of BD patients (2014 ICBD criteria).
- ANCA-positive BD patients showed significantly higher rates of vascular manifestations, especially in upper extremities and visceral arteries.
- ANCA positivity was not associated with adverse outcomes like mortality, acute coronary syndrome, or deep vein thrombosis.
Conclusions:
- ANCA positivity in BD is associated with specific vascular involvement at diagnosis.
- ANCA positivity is not a predictor of poor prognosis or adverse outcomes in BD patients.
- Further research may clarify the role of ANCA in the vascular complications of BD.
Purpose:
We investigated whether antineutrophil cytoplasmic antibody (ANCA) positivity is associated with vascular manifestations at diagnosis of Behçet's disease (BD) and poor outcomes during follow-up.
Materials And Methods:
We retrospectively reviewed the medical records of 1060 patients with BD. Among them, 808 patients could be diagnosed with BD based on the revised version of the International Criteria for Behçet's Disease (ICBD) in 2014 (2014 ICBD criteria) and 588 patients could be diagnosed with BD based on the International Study Group (ISG) criteria proposed in 1990 (1990 ISG criteria). We examined the sites and patterns of vascular involvement in the BD patients at diagnosis and evaluated adverse outcomes during follow up, such as all-cause mortality, acute coronary syndrome, and deep vein thrombosis.
Results:
Among the 808 patients with BD based on the 2014 ICBD criteria, the rate of ANCA positivity at diagnosis was 2.2%. ANCA-positive BD patients exhibited a higher frequency of overall vascular manifestations (22.2% vs. 6.1%) and higher frequencies of vascular involvement in the upper extremities and visceral arteries than ANCA-negative BD patients (5.6% vs. 0.1% and 5.6% vs. 0.1%). Among the 588 BD patients based on the 1990 ISG criteria, similarly, ANCA-positive BD patients exhibited a higher frequency of vascular manifestations than ANCA-negative BD patients. ANCA positivity, however, did not seem to be associated with poor outcomes in BD patients during follow up.
Conclusion:
ANCA positivity in BD patients was found to be associated with cross-sectional vascular involvement in the upper extremities and visceral arteries at diagnosis but was not predictive of poor outcomes during follow-up.
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