Idiopathic Progressive Tracheobronchial Stenosis of 20 Years' Duration: Response to Anti-inflammatory Treatment

Abstract

We report a unique case of progressive tracheobronchial stenosis in a 52-year-old woman who presented to us with stridor and dyspnea at rest. Her initial symptoms began 20 years earlier, at which time subglottic stenosis of ill-defined etiology necessitated tracheal resection with end-to-end anastomosis. Tracheal biopsy at the time revealed nonspecific inflammation without granulomas, vasculitis, infection, amyloidosis, or malignancy. Over subsequent years, she underwent multiple endobronchial laser resections of the trachea for recurrent disease. On presentation to us, flexible bronchoscopy showed inflammatory stenoses of the left mainstem bronchus and bronchus intermedius. Bronchial biopsy showed acute and chronic stromal inflammation with scattered plasma cells and myofibroblasts against a background of dense fibrosis. Review of the initial tracheal resection specimens and subsequent bronchial specimens revealed areas of high collagenous content with a relatively scant overall myofibroblastic cellular infiltrate; stains for S-100 and anaplastic lymphoma kinase were negative. A diagnosis of idiopathic tracheal stenosis was made with unusual accompanying bronchial involvement, that is, idiopathic tracheobronchial stenosis. Inflammatory airway bronchostenoses were stabilized by high-dose steroids followed by weekly methotrexate therapy, as evidenced by serial flexible bronchoscopies and sequential chest computed tomography with 3-dimensional reconstruction imaging. To our knowledge, this is the first reported case of combined idiopathic tracheal and bronchial stenosis stabilized with anti-inflammatory treatment.