UNASSIGNED: Tracheobronchial stenoses (TBSs) are potentially severe manifestations of @entity2883 with @entity2883 (Wegener's) (GPA) that usually respond poorly to @entity288 and immunosuppressive agents. We describe 26 GPA @entity1 with >= 1 tracheal (mainly subglottic, @entity9313 ) and/or @entity850 (ses) (BS(s)).Sixteen @entity1 had solitary @entity9313 and 10 had >= 1 BS(s). The male/female sex ratio was 9:17, and the median age at GPA diagnosis was 32 years (3:13 and 28 years, respectively, for @entity9313 patients). Antineutrophil cytoplasm antibodies were proteinase 3-positive in 65.5% of the @entity1 (50% of those with @entity9313 ).Despite conventional GPA therapy, 62% @entity1 experienced >= 1 stenosis relapse(s) (81% of @entity9313 @entity1 , for a total of 1-8 relapses per @entity1 ). None of the several systemic or endoscopic treatments prevented future relapses. @entity786 induction therapy was effective in 4/6 @entity1 with BS(s) and in 1 @entity1 with @entity9313 among the 7 treated. After many relapses, rituximab achieved remission in 3/4 @entity9313 @entity1 . Endoscopic treatments (dilation, laser, @entity228 injection, etc.) had only transient efficacy. Other GPA manifestations relapsed independently of TBSs. One @entity9313 @entity1 died of acute @entity649 .Our findings confirmed that TBSs are severe GPA manifestations that evolve independently of other organ involvements and do not respond to conventional systemic regimens. As previously described, our population was younger and comprised more females than usual GPA @entity1 , especially those with @entity9313 .The small number of @entity1 and the wide variety of local and systemic treatments prevent us from drawing definitive conclusions about the contribution of each procedure. However, @entity786 seemed to effectively treat BSs, but not @entity9313 , and rituximab may be of interest for @entity9313 management.

Tracheobronchial Stenoses in XXXX With @entity2883 (Wegener's): A Report on 26 Cases.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity9313 :: ('MESH:C536632', 'Disease') :: ['SGS'] @entity649 :: ('MESH:D012128', 'Disease') :: ['respiratory distress syndrome'] @entity786 :: ('MESH:D003520', 'Chemical') :: ['cyclophosphamide', 'Cyclophosphamide'] @entity228 :: ('CHEBI:50858', 'Chemical') :: ['corticosteroid'] @entity2883 :: ('MESH:D014890', 'Disease') :: ['polyangiitis', 'granulomatosis'] @entity850 :: ('MESH:D003251', 'Disease') :: ['bronchial stenosis'] @entity288 :: ('MESH:D000305', 'Chemical') :: ['corticosteroids']
@entity2883 :: (MESH:D014890,Disease) :: ['Granulomatosis']
