Endobronchial involvement is a common feature of sarcoidosis. The mucosa may appear normal or there may be inflammatory changes with erythematous lesions, miliary nodules, and, rarely, mass-like lesions leading to obstruction. In patients with bronchial lesions, the bronchial biopsy may be positive in three-quarters of patients. On the other hand, granulomas may be identified in one-third of patients with a normal mucosa. Current data on endobronchial sarcoidosis is primarily associated with the treatment and prognosis of obstructive mass-like granulomas. Data relevant to clinical features of superficial endobronchial involvement and its implications on prognosis and extrapulmonary organ involvement is lacking. The results of our two previous studies reveal that clinical findings of endobronchial disease may be different. These studies also suggest that there is a great difference between patients with no endobronchial involvement, limited and diffuse endobronchial disease in regard to clinical features, and prognosis. The incidence of extrapulmonary organ involvement is also significantly different between the three groups. In this short review, the clinical findings of endobronchial sarcoidosis have been defined, the effects of superficial endobronchial involvement on prognosis, and extrapulmonary organ involvement.
Sarcoidosis, endobronchial, limited, diffuse, pulmonary, prognosis
Cuneyt Tetikkurt, MD, declares no conflicts of interest in relation to this article. No funding was received for the publication of this article.
September 07, 2015 Accepted
September 22, 2015
Cuneyt Tetikkurt, MD, Pulmonary Diseases Department, Cerrahpasa Medical Faculty, Istanbul University, 34098 Cerrahpasa-Fatih/Istanbul, Turkey. E: firstname.lastname@example.org
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Sarcoidosis is a chronic systemic disease of unknown origin that is characterized by the formation of noncaseating granulomas in various organs, predominantly in the lungs. Granulomas are the pathologic hallmark of the disease and usually occur in the bronchial submucosa allowing bronchoscopic diagnosis by the use of a variety of diagnostic modalities to assess the airway in sarcoidosis.1,2 The bronchial mucosa in sarcoidosis may appear normal, inflammation with miliary or large nodules containing noncaseating granulomas may be present, and, less commonly, yellowish nodules with a cobblestone appearance may be observed.2,3 Occasionally, endobronchial granulomas form a mass-like lesion that can obstruct the bronchial lumen.4
Endobronchial involvement is common and granulomas are found anywhere in the respiratory tract with positive endobronchial biopsy findings in up to 40 to 70 % of patients.3–7 Bronchial biopsy may identify granulomas in approximately a third of cases with a normal mucosa because the granulomas are usually located in the airways. In the presence of mucosal lesions, diagnostic yield of bronchoscopic biopsies may be as high as 75 %.3–6
Although endobronchial disease is frequent in sarcoidosis, data concerning the clinical characteristics and prognosis of such patients is missing. The clinical features and prognosis of endobronchial disease have not been reported in the literature. Data relevant to patients with endobronchial sarcoidosis are limited with proximal bronchial involvement leading to bronchial stenosis or narrowing. The prognostic implications of endobronchial disease have only been assessed as a determinant of a factor causing stenosis.5,7
In this review, the main aim is to discuss the clinical features of endobronchial disease in regard to prognosis and extrapulmonary organ involvement in sarcoidosis.
Endobronchial Involvement in Sarcoidosis
Endobronchial involvement is not an uncommon sequela of sarcoidosis. Bronchoscopy can provide valuable information regarding the type, extent, and location of endobronchial disease for visible lesions and thereby allows histopathologic confirmation. Even when the airway mucosa appears normal, the diagnostic yield of bronchial biopsy may reach to 30 %.
Sarcoidosis confined to the airway may present with cough, wheeze, and dyspnea out of proportion to the parenchymal radiologic changes.8 Bronchial stenosis or narrowing are more frequently detected later in the course of the disease, mainly affecting the proximal parts of the
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Sarcoidosis, endobronchial, limited, diffuse, pulmonary, prognosis