AIMS: @entity1 with @entity1614 have an increased risk of life-threatening aortic complications, mostly preceded by @entity384 . In the FBN1C1039G/+ @entity1614 @entity19 model, @entity9886 decreases @entity384 . We recently confirmed this beneficial effect of @entity9886 in adult @entity1 with @entity1614 . The straightforward translation of this @entity19 model to @entity1 is reassuring to test novel treatment strategies. A number of studies have shown signs of @entity32 in aortic tissue of @entity1614 @entity1 . This study examined the efficacy of anti-inflammatory therapies in attenuating @entity384 in @entity1614 and compared effects to the main preventative agent, @entity9886 . METHODS AND RESULTS: To inhibit @entity32 in FBN1C1039G/+ @entity1614 @entity19 , we treated the @entity19 with @entity9886 (angiotensin II receptor type 1 inhibitor), @entity1667 ( @entity228 ) or abatacept (T-cell-specific inhibitor). Treatment was initiated in adult @entity1614 @entity19 with already existing @entity384 , and applied for eight weeks. @entity1667 - or abatacept-treated @entity19 did not reveal a reduction in @entity384 . In this short time frame, @entity9886 was the only treatment that significantly reduced aorta @entity32 , transforming growth factor-beta (TGF-b) signaling and @entity384 rate in these adult @entity1614 @entity19 . Moreover, the @entity1667 -treated @entity19 had significantly more aortic alcian blue staining as a marker for aortic damage. CONCLUSION: Anti-inflammatory agents do not reduce the @entity384 rate in @entity1614 @entity19 , but possibly increase aortic damage. Currently, the most promising therapeutic drug in @entity1614 is @entity9886 , by blocking the angiotensin II receptor type 1 and thereby inhibiting pSmad2 signaling.

No beneficial effect of general and specific anti-inflammatory therapies on @entity384 in marfan XXXX .

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients', 'man'] @entity32 :: ('MESH:D007249', 'Disease') :: ['inflammation'] @entity1667 :: ('MESH:D008775', 'Chemical') :: ['methylprednisolone', 'Methylprednisolone'] @entity19 :: ('10090', 'Species') :: ['mice', 'mouse'] @entity384 :: ('MESH:D002311', 'Disease') :: ['aortic root dilatation', 'aortic root dilation', 'aortic dilatation'] @entity1614 :: ('MESH:D008382', 'Disease') :: ['Marfan', 'Marfan syndrome'] @entity228 :: ('CHEBI:50858', 'Chemical') :: ['corticosteroid'] @entity9886 :: ('MESH:D019808', 'Chemical') :: ['losartan']
@entity19 :: (10090,Species) :: ['mice']
