• Jennifer Catano, Yurdagul Uzunhan, Romain Paule, Jérémie Dion, Alexis Régent, Paul Legendre, François Gonin, Emmanuel Martinod, Pascal Cohen, Xavier Puéchal, Véronique Le Guern, Luc Mouthon, André Coste, Christine Lorut, Candice La Croix, Sophie Périé, and Benjamin Terrier.
• Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP)-Centre Université Paris (CUP), Paris, France.
• Chest. 2022 Jan 1; 161 (1): 257-265.

BackgroundSubglottic stenosis (SGS) and tracheal stenosis (TS) are characterized by a narrowing of the airways. The goal of this study was to describe the characteristics and prognosis of nontraumatic and nontumoral SGS or TS.Research QuestionWhat are the inflammatory etiologies of SGS and TS, and what are their characteristics and prognosis?Study Design And MethodsThis multicenter, observational retrospective study was performed in patients with SGS or TS that was neither traumatic nor tumoral.ResultsEighty-one patients were included, 33 (41%) with granulomatosis with polyangiitis (GPA) and 21 (26%) with relapsing polychondritis (RP). GPA-related stenoses exhibited circumferential subglottic narrowing in 85% of cases, without calcifications. In contrast, RP-related stenoses displayed anterior involvement in 76%, in a longer distance from vocal cords (4 cm), with calcifications in 62%, and extension to bronchi in 86%. Other diagnoses included bullous dermatoses (n = 3), amyloidosis (n = 3), sarcoidosis (n = 2), and Crohn's disease (n = 2); the remaining stenoses (n = 15) were idiopathic. SGS/TS was the initial manifestation of the disease in 66% of cases, with a median interval from stenosis to disease diagnosis of 12 months (interquartile range, 0-48 months). Despite the use of glucocorticoids in 80%, combined with methotrexate in 49%, endoscopic procedures were required in 68% of patients. Relapses of stenoses occurred in 76% without any difference between causes (82% in GPA, 67% in RP, and 75% in idiopathic SGS/TS). Three patients died due to the stenosis, two of RP and one of GPA.InterpretationThese data show that GPA and RP are the two main inflammatory diseases presenting with SGS/TS. GPA-related stenoses are mostly subglottic and circumferential, whereas RP-related stenoses are mostly tracheal, anterior, and calcified with a frequent extension to bronchi. Relapses of stenoses are common, and relapse rates do not differ between causes. Diagnosis and management of SGS/TS require a multidisciplinary approach.Copyright © 2021 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

46 keywords...

hide…