• Charlotte Girard, Pierre Charles, Benjamin Terrier, Guillaume Bussonne, Pascal Cohen, Christian Pagnoux, Vincent Cottin, Jean-François Cordier, and Loïc Guillevin.
• From the Department of Internal Medicine (CG, PaC, BT, GB, LG), National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital; INSERM U1060 (CG, PaC, BT, GB, LG), Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, University of Paris 5-René-Descartes, Paris; Department of Internal Medicine (CG), Department of Rheumatology, Mount Sinaï Hospital, Toronto, Ontario, Canada (CP), Edouard-Herriot University Hospital, Lyon; National Referral Center for Rare Pulmonary Diseases (VC, J-FC), Louis-Pradel Hospital, Lyon, France; and Department of Internal Medicine (PiC), Institut Mutualiste Montsouris, Paris.
• Medicine (Baltimore). 2015 Aug 1; 94 (32): e1088.

AbstractTracheobronchial stenoses (TBSs) are potentially severe manifestations of granulomatosis with polyangiitis (Wegener's) (GPA) that usually respond poorly to corticosteroids and immunosuppressive agents. We describe 26 GPA patients with ≥1 tracheal (mainly subglottic, SGS) and/or bronchial stenosis(ses) (BS(s)).Sixteen patients had solitary SGS 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 SGS patients). Antineutrophil cytoplasm antibodies were proteinase 3-positive in 65.5% of the patients (50% of those with SGS).Despite conventional GPA therapy, 62% patients experienced ≥1 stenosis relapse(s) (81% of SGS patients, for a total of 1-8 relapses per patient). None of the several systemic or endoscopic treatments prevented future relapses. Cyclophosphamide induction therapy was effective in 4/6 patients with BS(s) and in 1 patient with SGS among the 7 treated. After many relapses, rituximab achieved remission in 3/4 SGS patients. Endoscopic treatments (dilation, laser, corticosteroid injection, etc.) had only transient efficacy. Other GPA manifestations relapsed independently of TBSs. One SGS patient died of acute respiratory distress syndrome.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 patients, especially those with SGS.The small number of patients and the wide variety of local and systemic treatments prevent us from drawing definitive conclusions about the contribution of each procedure. However, cyclophosphamide seemed to effectively treat BSs, but not SGS, and rituximab may be of interest for SGS management.

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