Tracheal Stenosis Due to Sarcoidosis

Abstract

To the Editor: Stenotic involvement of the large airways is an unusual complication of sarcoidosis, with bronchial stenosis recently being described.1Westcott JL Noehren JH Bronchial stenosis in chronic sarcoidosis.Chest. 1973; 73: 893-897Crossref Scopus (24) Google Scholar, 2Olsson T Gjornstad-Pettersen H Stjernberg NL Bronchostenosis due to sarcoidosis.Chest. 1979; 75: 663-667Abstract Full Text Full Text PDF PubMed Scopus (65) Google Scholar To our knowledge we report the first case of tracheal stenosis due to sarcoidosis. A 48-year-old black woman was referred to Massachusetts General Hospital for surgical repair of tracheal stenosis due to sarcoidosis. Initial complaints of a decrease in voice strength 30 years prior to admission prompted a non-diagnostic evaluation by a local physician. One year later, skin lesions appeared on her extremities which were biopsied and revealed non-caseating granulomas consistent with sarcoidosis. She received a course of corticosteroids with improvement of her skin lesions but no change in her diminished voice strength. Twenty-five years prior to admission, a chest roentgenogram revealed a diffuse interstitial pattern with nodules. Ten years later, her chest roentgenogram appeared unchanged, but she continued to experience decreasing strength of her voice, and three years prior to admission she experienced her first episode of stridulent breathing. She was only minimally improved with antibiotics and a short course of beclomethasone and systemic corticosteroids. Two years prior to admission, her chest roentgenogram revealed emphysematous changes and bullae in addition to a diffuse interstitial pattern. Tracheal tomograms revealed narrowing of the trachea. Her pulmonary function tests were consistent with an obstructive process. Over the next one and a half years prior to admission she had progressive stridor becoming more difficult to manage with antibiotics and bronchodilators. On admission to our hospital additional history obtained was negative for smoking, asthma, previous bronchoscopy, or intubation. Physical examination revealed minimal inspiratory stridor. Her skin tests were positive for mumps and negative for PPD. Results of laboratory tests and arterial blood gases were essentially normal. Pulmonary function tests again revealed severe obstructive lung disease. Tracheal tomograms revealed more severe narrowing of the trachea and left mainstem bronchus when compared to films two years earlier (Fig 1). During bronchoscopy, an extensive 5-6 mm narrowing of the trachea was observed and tracheal dilatation was performed. Her inspiratory stridor improved and she was discharged on a prolonged course of corticosteroids and bronchodilators with repeated tracheal dilatations anticipated in the future. Sputum cultures for mycobacteria and fungi remained free of growth for over a three-month period. Tracheal involvement due to sarcoidosis has been limited to the description of tracheal dystonea, a weakening of the membranous wall.3Ellefsen P Tracheal dystonea and sarcoidosis.Acta Otolaryng. 1979; 70: 438-442Crossref Scopus (4) Google Scholar Our patient experienced at least a 30-year history of sarcoidosis with symptoms initially of laryngeal involvement. In the three years prior to admission progressive tracheal stenosis developed manifested by inspiratory stridor and dyspnea, with serial tracheal tomograms demonstrating progressive stenosis of the trachea. This entity appears distinct from tracheal dystonea and may represent a form of end-stage tracheomalacia where the trachea loses its supporting elastic and cartilagenous structures.4Campbell AH Definition and causes of the tracheobronchial collapse syndrome.Br J Dis Chest. 1966; 61: 1-11Abstract Full Text PDF Google Scholar Unlike reported cases of bronchial stenosis due to sarcoid, which has been noted to arrest following treatment with high dose corticosteroids,2Olsson T Gjornstad-Pettersen H Stjernberg NL Bronchostenosis due to sarcoidosis.Chest. 1979; 75: 663-667Abstract Full Text Full Text PDF PubMed Scopus (65) Google Scholar our patient with tracheal stenosis failed to stabilize and deteriorated in spite of several short courses of systemic corticosteroids. Therapy in these patients may be limited to prolonged courses of corticosteroids combined with repeated tracheal dilatations.