XXXX Complicating Fibrosing @entity1764 .

@entity1 @entity1143 @entity120 @entity1764 @entity228 @entity2556 @entity358 @entity370 @entity378 @entity288
Fibrosing @entity1764 is caused by a proliferation of fibrous tissue in the mediastinum with encasement of mediastinal viscera and compression of mediastinal bronchovascular structures. @entity120 ( @entity120 ) is a severe complication of fibrosing @entity1764 caused by extrinsic compression of the pulmonary arteries and/or veins.We have conducted a retrospective observational study reviewing clinical, functional, hemodynamic, radiological characteristics, and outcome of 27 consecutive cases of @entity120 associated with fibrosing @entity1764 diagnosed between 2003 and 2014 at the French Referral Centre for @entity120 .Fourteen @entity1 and 13 @entity1 with a median age of 60 years (range 18-84) had @entity120 confirmed on right heart catheterization. The causes of fibrosing @entity1764 were @entity370 (n=13), @entity378 confirmed or suspected (n=9), mediastinal irradiation (n=2), and idiopathic (n=3). Sixteen @entity1 (59%) were in NYHA functional class III and IV. Right heart catheterization confirmed moderate to severe @entity120 with a median mean pulmonary artery pressure of 42 mm Hg (range 27-90) and a median cardiac index of 2.8 L/min/m (range 1.6-4.3). Precapillary @entity120 was found in 22 @entity1 , postcapillary @entity120 in 2, and combined postcapillary and precapillary @entity120 in 3. Severe extrinsic compression of pulmonary arteries (>60% reduction in diameter) was evidenced in 2, 8, and 12 @entity1 at the main, lobar, or segmental levels, respectively. Fourteen @entity1 had at least one severe @entity358 with associated @entity1143 in 6 of them. @entity2556 therapy was initiated in 7 @entity1 and corticosteroid therapy (0.5-1mg/kg/day) was initiated in 3 @entity1 with @entity370 , with 9 other being already on low-dose @entity288 . At 1-year follow-up, 3 @entity1 had died and among the 21 @entity1 evaluated, 3 deteriorated, 14 were stable, and only 4 @entity1 with @entity370 improved (4 receiving corticosteroids and 1 receiving corticosteroids and @entity2556 therapy). Survival was 88%, 73%, and 56% at 1, 3, and 5 years, respectively.We found no clear clinical improvement with the use of specific @entity2556 therapy. @entity228 therapy may be associated with clinical improvement, in some @entity1 with fibrosing @entity1764 due to @entity370 . Although never performed for this indication, lung transplantation may be proposed in eligible @entity1 with severe @entity120 and fibrosing @entity1764 .
