Management of Laryngotracheobronchial Sequelae and Complications of Relapsing Polychondritis

Abstract

Relapsing polychondritis is a rare multisystem disorder of unknown etiology characterized by recurrent inflammation and degeneration of cartilage and connective tissue. Laryngotracheobronchial complications are the most severe manifestations of the disease and present the most challenging management decisions. We present four cases of relapsing polychondritis with laryngotracheobronchial manifestations that illustrate the clinical features and review the treatment options.

Three Cases of Relapsing Polycondritis with Isolated Laryngotracheal Stenosis

“…There were histopathologic diagnoses of airway chondritis that were giving symptoms only through LTS in all of our cases. In addition, fiberoptic or direct endoscopic examination, CT, magnetic resonance imaging, cine tracheography, and laryngotracheograms are very helpful in detecting stenotic segment width and localization in LTS diagnosis (6). Both direct laryngoscopy and CT examination were performed for the diagnosis in the adult cases presented in this study.…”

“…If laryngotracheal, bronchial, cardiovascular, renal, ocular, and neurological involvement is seen, immunosuppressants such as glucocorticoids, dapsone, cyclophosphamide, azathioprine, cyclosporine, and methotrexate, which are more aggressive treatment choices, can be used. Elective surgical interventions are corticosteroid injections, recurrent dilatations, stenting, tracheotomy, Montgomery T-tube placement, and tracheal resection with end-to-end anastomosis (6). In this study, medical treatment was initiated in the first case without severe airway obstruction and without tracheotomy.…”

“…In LTS, edema develops in the airway during the active period of the disease, and fibrous tissue develops due to chronic inflammation in the late period and the airway collapses due to the destruction of the laryngotracheal cartilages (6). Lung infections develop more frequently secondary to obstruction, which occurs as a result of infection and scarring (6). While the auricle (83%) is classically affected most commonly in adults, the larynx and trachea (50%), nasal, costal cartilage, joints, eye (20-60%), skin (36%), and heart (<%10) are other anatomical structures that can be affected (4).…”

“…Although today, it is thought that there is a genetic transmission, familial inheritance still remains uncertain (5). Specific antibodies and triggered immunological responses against type 2 collagen that develop in two-thirds of patients are thought to be responsible for the pathogenesis of RP (6). At the same time, additional autoimmune, rheumatologic, and hematologic pathologies are observed in 30% of patients (7).…”

Iatrogenic Membranous Tracheal Tear in the Setting of Relapsing Polychondritis

“…Recurrent inflammation of the cartilaginous trachea leads to decreased overall support and increased propensity for collapse due to negative intrathoracic pressures [4]. When severe, these sequelae can be managed with repeated dilation, stenting, laryngotracheal reconstruction, and rarely tracheostomy [2][3][4][5]. Prospective data regarding medical management is sparse and RP is currently treated with a combination of corticosteroids, methotrexate, azathioprine, and newer biologic agents [6].…”