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Báo cáo y học: "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"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học Wertheim cung cấp cho các bạn kiến thức về ngành y đề tài: 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 et al. Journal of Cardiothoracic Surgery 2010 5 66 http www.cardiothoracicsurgery.Org content 5 1 66 JOTS JOURNAL OF CARDIOTHORACIC SURGERY CASE REPORT Open Access 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 1.2t I- 1t 3 2 1 Gabriele Weissen-Plenz 1 Omer Sezer Christian Vahlhaus Horst Robenek Andreas Hoffmeier Tonny DTTjan1 Hans H Scheld1 Jurgen R Sindermann1 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. Background Cogan s syndrome is a rare disorder of unknown origin characterized by inflammatory ocular disease and vesti-buloauditory symptoms 1 2 . Major clinical features are interstitial keratitis and vestibuloauditory dysfunction. The variety of systemic manifestations includes fever splenomegaly lymphadenopathy and musculoskeletal complaints. Systemic vasculitis is found in about 10 of cases and may involve the large .