Characterization of the inflammatory cells in ascending thoracic aortic aneurysms in patients with Marfan syndrome, familial thoracic aortic aneurysms, and sporadic aneurysms.

Abstract

OBJECTIVE:

This study sought to characterize the inflammatory infiltrate in ascending thoracic aortic aneurysm in patients with Marfan syndrome, familial thoracic aortic aneurysm, or nonfamilial thoracic aortic aneurysm.

BACKGROUND:

Thoracic aortic aneurysms are associated with a pathologic lesion termed "medial degeneration," which is described as a noninflammatory lesion. Thoracic aortic aneurysms are a complication of Marfan syndrome and can be inherited in an autosomal dominant manner of familial thoracic aortic aneurysm.

METHODS:

Full aortic segments were collected from patients undergoing elective repair with Marfan syndrome (n = 5), familial thoracic aortic aneurysm (n = 6), and thoracic aortic aneurysms (n = 9), along with control aortas (n = 5). Immunohistochemistry staining was performed using antibodies directed against markers of lymphocytes and macrophages. Real-time polymerase chain reaction analysis was performed to quantify the expression level of the T-cell receptor beta-chain variable region gene.

RESULTS:

Immunohistochemistry of thoracic aortic aneurysm aortas demonstrated that the media and adventitia from Marfan syndrome, familial thoracic aortic aneurysm, and sporadic cases had increased numbers of T lymphocytes and macrophages when compared with control aortas. The number of T cells and macrophages in the aortic media of the aneurysm correlated inversely with the patient's age at the time of prophylactic surgical repair of the aorta. T-cell receptor profiling indicated a similar clonal nature of the T cells in the aortic wall in a majority of aneurysms, whether the patient had Marfan syndrome, familial thoracic aortic aneurysm, or sporadic disease.

CONCLUSION:

These results indicate that the infiltration of inflammatory cells contributes to the pathogenesis of thoracic aortic aneurysms. Superantigen-driven stimulation of T lymphocytes in the aortic tissues of patients with thoracic aortic aneurysms may contribute to the initial immune response.

• Authors: Rumin He, Dong-Chuan Guo, Wei Sun, Christina L Papke, Senthil Duraisamy, Anthony L Estrera, Hazim J Safi, Chul Ahn, L Maximilian Buja, Frank C Arnett, Jingwu Zhang, Yong-Jian Geng, Dianna M Milewicz
• Journal: The Journal of thoracic and cardiovascular surgery (J Thorac Cardiovasc Surg) Vol. 136 Issue 4 Pg. 922-9, 929.e1 (Oct 2008) ISSN: 1097-685X [Electronic] United States
• PMID: 18954631 (Publication Type: Comparative Study, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)

Chemical References

• Antigens, CD
• Antigens, Differentiation, T-Lymphocyte
• Biomarkers
• Receptors, Antigen, T-Cell
• Transforming Growth Factor beta2
• Vascular Cell Adhesion Molecule-1
• RNA

Topics

• Adult
• Aged
• Antigens, CD (immunology, metabolism)
• Antigens, Differentiation, T-Lymphocyte (genetics, metabolism)
• Aortic Aneurysm, Abdominal (immunology, pathology, surgery)
• Aortic Aneurysm, Thoracic (immunology, pathology, surgery)
• Biomarkers (analysis)
• Biopsy, Needle
• Cardiac Surgical Procedures (methods)
• Case-Control Studies
• Female
• Humans
• Immunohistochemistry
• Male
• Marfan Syndrome (genetics, pathology, surgery)
• Middle Aged
• Multivariate Analysis
• RNA (analysis)
• Receptors, Antigen, T-Cell (immunology, metabolism)
• Reference Values
• Reverse Transcriptase Polymerase Chain Reaction
• Sensitivity and Specificity
• Statistics, Nonparametric
• Tissue Culture Techniques
• Transforming Growth Factor beta2 (analysis, immunology)
• Vascular Cell Adhesion Molecule-1 (immunology, metabolism)