Full Papers
Clinicopathological characteristics of severe aortic valve regurgitation caused by Behçet’s syndrome
M. Zhang1, X. Wang2, Y. Liu3, X. Liu4, X. Yu5, L. Sun6, Z. Wang7, L. Zhang8, J. Liu9, G. Ma10, W. Chen11, W. Wang12, Q. Miao13, W. Zheng14
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Beijing, China.
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Beijing, China.
- Department of Cardiac Surgery, Peking Union Medical College Hospital, Dongcheng District, Beijing, China.
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Beijing, China.
- Department of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Beijing, China.
- Division of Infectious Diseases, Department of Internal Medicine, State Key Laboratory of Complex Severe and Rare Disease, Peking Union Medical College Hospital, Beijing, China.
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Beijing, China.
- Department of Cardiac Surgery, Peking Union Medical College Hospital, Dongcheng District, Beijing, China.
- Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. wwzvssxy@126.com
- Department of Cardiac Surgery, Peking Union Medical College Hospital, Dongcheng District, Beijing, China. miaoqipumc@hotmail.com
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Beijing, China. zhengwj@pumch.cn
CER18064
Full Papers
PMID: 40183327 [PubMed]
Received: 11/08/2024
Accepted : 21/01/2025
In Press: 02/04/2025
Abstract
OBJECTIVES:
Aortic valve regurgitation (AR) caused by Behçet’s syndrome (BS) has high mortality. Preoperative biologics reduced systemic inflammation, but their effect on lesion inflammation remains unclear.
METHODS:
Twenty-two BS patients with severe AR who underwent cardiac surgery with retained pathological specimens were included. The pathology of the aortic wall and/or valve was re-analysed based on their preoperative disease activity and treatment strategy. Immunohistochemistry (IHC) assessed the distribution of CD4+, CD8+, CD20+ and CD68+ cells.
RESULTS:
The mean diagnosis age was 39.6±13.1 years, with a median disease duration of 9 (3-35) years. Seven (31.8%) underwent cardiac surgery during the active phase due to uncontrollable disease progression, while 15 (68.2%) were in remission. Pathologically, severe AR caused by BS is characterised by mixed inflammatory cell infiltration in the aortic wall. Active cases showed significantly more diffuse infiltration of CD4+ (100% vs. 8.3%, p=0.0002) and CD8+ (71.4% vs. 20%, p=0.058) T cells in the aortic adventitia, with more neutrophil infiltration in the aortic valve (60% vs. 7.7%, p=0.044). Notably, less CD68+ macrophage infiltration (57.2% vs. 0%, p=0.045), CD4+ T cell diffusion (57.1% vs. 0%, p=0.045), and vasa vasorum mucoid degeneration (85.7% vs. 20%, p=0.017) were observed in the aortic adventitia of patients receiving preoperative biologics, together with less aortic valve necrosis (71.4% vs. 0%, p=0.023).
CONCLUSIONS:
Overall, our study provides valuable insights into the pathology of severe AR caused by BS as a mixed inflammatory infiltration and provides the first pathological rationale for achieving preoperative remission and early biologics to improve the prognosis.
