Chest
Volume 136, Issue 5, November 2009, Pages 1371-1380
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Special Feature
Airway Involvement in Sarcoidosis

https://doi.org/10.1378/chest.08-2569Get rights and content

Sarcoidosis is a common disease and affects the respiratory system in > 90% of cases, most commonly the intrathoracic lymph nodes and the respiratory parenchyma. Less commonly, the airways are involved, and the disease is manifested as mucosal erythema, edema, granularity and cobblestoning, plaques, nodules, and bronchial stenosis, airway distortion, traction bronchiectasis, and bronchiolitis. Airway involvement may lead to airflow limitation. Involvement of oral, nasal, and pharyngeal mucosa may cause hoarseness, dysphagia, laryngeal paralysis, and upper airway obstruction. Airway symptoms are important indicators of airway involvement in sarcoidosis. Pulmonary function testing, radiologic imaging, and bronchoscopy occupy a significant role in the diagnosis and management of airway involvement in patients with sarcoidosis.

Section snippets

Airway Involvement

The mechanisms responsible for the airway involvement are diverse.11, 12, 13, 16, 21, 22, 23, 24, 25 The entire spectrum of the airways from the nasal and oral passages to terminal bronchioles can be affected by sarcoidosis (Table 1). The anatomic abnormalities caused by sarcoidosis may or may not result in pulmonary symptoms or physiologic abnormalities. Indeed, the pulmonary function testing may show normal results even when anatomic changes documented by imaging studies are severe. The

Supraglottic Airways

Nasal passages, oropharynx, supraglottic structures, and the larynx develop sarcoid granulomas in approximately 6% of patients with sarcoidosis.5, 26, 27, 28, 29, 30, 31 Sarcoid lesions can occur in nasal and oral mucosa, occasionally with ulceration; anosmia improves after steroid therapy.32, 33, 34, 35 An epidemiologic study28 of 736 patients with sarcoidosis in the United States observed ear, nose, and throat involvement in 22 patients (2.3%). Another study36 of 818 patients with multisystem

Larynx

Laryngeal sarcoidosis often occurs as an isolated phenomenon and is usually attributed to asthma.44, 45, 46, 47 Occasionally, laryngeal sarcoid can lead to progressive life-threatening airway obstruction.39, 45 Unless the index of suspicion is high and appropriate biopsy specimens are obtained, the diagnosis can be missed, because, even in patients with multisystemic sarcoidosis, the occurrence of laryngeal sarcoidosis is uncommon.48 The incidence of laryngeal sarcoidosis is estimated to be

Central Airways

The trachea and main bronchi are less frequently affected than the lobar, segmental, subsegmental, and distal airways. Sarcoid granulomas of trachea, main carina, and major bronchi by themselves seldom produce significant obstructive symptoms or airway dysfunction.21, 23, 53, 54 Cough is the main symptom. Symptoms, clinical examination, flow-volume curves, and bronchoscopy help in assessing the severity of the central airway stenosis (Fig 1).55

Mainstem bronchial stenosis as well as segmental

Distal Airways

Lobar, segmental, subsegmental, and more distal bronchi as well as bronchioles are affected by sarcoidosis, which is manifested as mucosal inflammation, endobronchial granulomas, stenosis, extrinsic compression, distortion, bronchiectasis, bronchiolitis, airway hyperreactivity, and streaky hemoptysis. These can lead to airway dysfunction and respiratory symptoms. Sarcoid granulomas tend to develop along the bronchovascular bundle or in the vicinity of the airways. All of these changes are more

Effect of Tobacco Smoking

Data from several publications on the effect of smoking in patients with sarcoidosis are controversial. Both a high incidence of small airway disease in patients with sarcoidosis who are smokers as well as a synergism between sarcoidosis and smoking have been reported.12, 84 A negative association between sarcoidosis and smoking,85 as well as the absence of any relationship between these two factors, have been reported.86

Airflow Function Testing

The precise incidence of airway dysfunction in sarcoidosis is difficult to estimate. An obstructive pattern on spirometric testing has been reported in 4% to 75% of patients.10, 13, 28, 55, 65, 87 Obstructive airway dysfunction is the result of advanced parenchymal fibrotic disease leading to airway distortions, bullous changes, and peribronchial or peribronchiolar fibrosis.13, 17, 88, 89 Obstructive airway disease has been noted in 63% of the African-American patients with sarcoidosis compared

Airway Imaging

Chest roentgenographic abnormalities are frequently the first indication of sarcoidosis, even in asymptomatic patients. Subtle airway involvement is not visible on plain chest films. Secondary traction bronchiectasis may be suggested by the prominent air bronchograms. High-resolution CT scans may demonstrate bronchial distortion, angulation, and displacement (Fig 6). Decreased airway luminal diameter caused by endobronchial granulomas and evidence of bronchial mural thickening may be seen.19, 93

Bronchoscopy

Bronchoscopy plays a significant role in the diagnosis and management of airway sarcoidosis. The role of the technique in the retrieval of immune effector cells and infectious organisms by analyzing BAL fluid, biopsy specimens of pulmonary parenchymal sarcoid, and needle aspiration/biopsy samples of enlarged lymph nodes in the mediastinum and hilar regions is well known.97 These bronchoscopic techniques have an important role in the diagnosis of infectious diseases in sarcoid patients in whom

Differential Diagnosis

Endobronchial abnormalities similar to those encountered in sarcoidosis occur in patients with bronchitis, Wegener granulomatosis, mediastinal granuloma and fibrosis, histoplasmosis, blastomycosis, coccidioidomycosis mycobacterioses, syphilis, actinomycosis, malignant neoplasms, cartilaginous tumors, amyloidosis, papillomatoses, nonspecific mucosal granulomas, inflammatory bowel disorders, and radiation-induced mucositis. Clinicopathologic correlation is essential to exclude nonsarcoid

Treatment

Published data provide insufficient evidence-based data to recommend a standardized approach to treat airway sarcoidosis. As a result, definitive recommendations cannot be provided for treatment. Currently, the decision to undertake empirical therapy is based on the severity and rate of progression of airway symptoms. The presence of airway dysfunction, as shown by pulmonary function testing, may not require treatment if airway-related symptoms are absent. If airway involvement is present as

Acknowledgments

Financial/nonfinancial disclosures: The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

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