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.
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.


METHODS
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).


CONCLUSIONS
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.
Weissen-Plenz
Gabriele
G
Department of Thoracic and Cardiovascular Surgery, University Hospital of Muenster, Albert-Schweitzer-Strasse 33, 48149 Muenster, Germany.
Sezer
Omer
O
Vahlhaus
Christian
C
Robenek
Horst
H
Hoffmeier
Andreas
A
Tjan
Tonny D T
TD
Scheld
Hans H
HH
Sindermann
Jürgen R
JR
D000784:Aortic Dissection / Q000209:etiology / Q000276:immunology / Q000503:physiopathology* / Q000601:surgery; D001011:Aorta / Q000276:immunology / Q000503:physiopathology*; D001014:Aortic Aneurysm / Q000209:etiology / Q000276:immunology / Q000503:physiopathology* / Q000601:surgery; D001808:Blood Vessels / Q000276:immunology / Q000503:physiopathology; D055952:Cogan Syndrome / Q000150:complications*; D005260:Female; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000276:immunology*; D006801:Humans; D020782:Matrix Metalloproteinases / Q000276:immunology; D008875:Middle Aged; D032389:Myocytes, Smooth Muscle / Q000276:immunology*
D016178:Granulocyte-Macrophage Colony-Stimulating Factor; D020782:Matrix Metalloproteinases