Limits...
Aortic dissection associated with Cogans's syndrome: deleterious loss of vascular structural integrity is associated with GM-CSF overstimulation in macrophages and smooth muscle cells.

Weissen-Plenz G, Sezer O, Vahlhaus C, Robenek H, Hoffmeier A, Tjan TD, Scheld HH, Sindermann JR - J Cardiothorac Surg (2010)

Bottom Line: Systemic vasculitis is found in about 10% of cases.Remarkably, exceeding matrix deterioration was associated with massively increased levels of granulocyte macrophage colony stimulating factor (GM-CSF).Our data suggest that the persistently increased secretion of the inflammatory mediator GM-CSF by resident inflammatory cells but also by SMC may be the trigger of aortic wall structural deterioration.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Thoracic and Cardiovascular Surgery, University Hospital of Muenster, Albert-Schweitzer-Strasse 33, 48149 Muenster, Germany.

ABSTRACT

Background: Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory ocular disease and vestibuloauditory symptoms. Systemic vasculitis is found in about 10% of cases.

Case presentation: A 46-year-old female with Cogans's syndrome and a history of arterial hypertension presented with severe chest pain caused by an aneurysm of the ascending aorta with a dissection membrane located a few centimeters distal from the aortic root. After surgery, histopathological analysis revealed that vascular matrix integrity and expression of the major matrix molecules was characterized by elastolysis and collagenolysis and thus a dramatic loss of structural integrity. Remarkably, exceeding matrix deterioration was associated with massively increased levels of granulocyte macrophage colony stimulating factor (GM-CSF).

Conclusion: Our data suggest that the persistently increased secretion of the inflammatory mediator GM-CSF by resident inflammatory cells but also by SMC may be the trigger of aortic wall structural deterioration.

Show MeSH

Related in: MedlinePlus

Transverse (A) and sagittal (B) CT views of the aorta. The dissection (arrows) is circumferential and begins distally to the coronary ostiae. arrows: dissection membrane
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC2933604&req=5

Figure 2: Transverse (A) and sagittal (B) CT views of the aorta. The dissection (arrows) is circumferential and begins distally to the coronary ostiae. arrows: dissection membrane

Mentions: A 46-year-old female with Cogans's syndrome and a history of arterial hypertension was admitted with severe chest pain. Briefly, physical examination revealed the absence of radial pulses and no measurable blood pressure of the upper extremities. Aortic angiography revealed an aortic arch syndrome with occlusion of both subclavian and vertrebral arteries (Fig. 1). Ultrasound showed a fusiform abdominal aortic aneurysm and axial computed tomography demonstrated an aneurysm of the ascending aorta with a dissection membrane located a few centimeters distal from the aortic root (Fig. 2). At surgery the ascending aorta showed severe signs of inflammation comprising reddish alterations of the aortic walls and a pattern of thickened as well as thinned wall areas of the aorta and adjacent vessels. The ascending aorta revealed a circumferential dissection distal from the coronary ostia associated with an overload of pastuous material. The patient underwent a replacement of the ascending aorta by a 28 mm VacscutekĀ® tube graft. After uneventful recovery the patient was discharged on postoperative day 17 with minimal and irrelevant neurological deficits.


Aortic dissection associated with Cogans's syndrome: deleterious loss of vascular structural integrity is associated with GM-CSF overstimulation in macrophages and smooth muscle cells.

Weissen-Plenz G, Sezer O, Vahlhaus C, Robenek H, Hoffmeier A, Tjan TD, Scheld HH, Sindermann JR - J Cardiothorac Surg (2010)

Transverse (A) and sagittal (B) CT views of the aorta. The dissection (arrows) is circumferential and begins distally to the coronary ostiae. arrows: dissection membrane
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2933604&req=5

Figure 2: Transverse (A) and sagittal (B) CT views of the aorta. The dissection (arrows) is circumferential and begins distally to the coronary ostiae. arrows: dissection membrane
Mentions: A 46-year-old female with Cogans's syndrome and a history of arterial hypertension was admitted with severe chest pain. Briefly, physical examination revealed the absence of radial pulses and no measurable blood pressure of the upper extremities. Aortic angiography revealed an aortic arch syndrome with occlusion of both subclavian and vertrebral arteries (Fig. 1). Ultrasound showed a fusiform abdominal aortic aneurysm and axial computed tomography demonstrated an aneurysm of the ascending aorta with a dissection membrane located a few centimeters distal from the aortic root (Fig. 2). At surgery the ascending aorta showed severe signs of inflammation comprising reddish alterations of the aortic walls and a pattern of thickened as well as thinned wall areas of the aorta and adjacent vessels. The ascending aorta revealed a circumferential dissection distal from the coronary ostia associated with an overload of pastuous material. The patient underwent a replacement of the ascending aorta by a 28 mm VacscutekĀ® tube graft. After uneventful recovery the patient was discharged on postoperative day 17 with minimal and irrelevant neurological deficits.

Bottom Line: Systemic vasculitis is found in about 10% of cases.Remarkably, exceeding matrix deterioration was associated with massively increased levels of granulocyte macrophage colony stimulating factor (GM-CSF).Our data suggest that the persistently increased secretion of the inflammatory mediator GM-CSF by resident inflammatory cells but also by SMC may be the trigger of aortic wall structural deterioration.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Thoracic and Cardiovascular Surgery, University Hospital of Muenster, Albert-Schweitzer-Strasse 33, 48149 Muenster, Germany.

ABSTRACT

Background: Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory ocular disease and vestibuloauditory symptoms. Systemic vasculitis is found in about 10% of cases.

Case presentation: A 46-year-old female with Cogans's syndrome and a history of arterial hypertension presented with severe chest pain caused by an aneurysm of the ascending aorta with a dissection membrane located a few centimeters distal from the aortic root. After surgery, histopathological analysis revealed that vascular matrix integrity and expression of the major matrix molecules was characterized by elastolysis and collagenolysis and thus a dramatic loss of structural integrity. Remarkably, exceeding matrix deterioration was associated with massively increased levels of granulocyte macrophage colony stimulating factor (GM-CSF).

Conclusion: Our data suggest that the persistently increased secretion of the inflammatory mediator GM-CSF by resident inflammatory cells but also by SMC may be the trigger of aortic wall structural deterioration.

Show MeSH
Related in: MedlinePlus