ABSTRACT
Aims: Airway involvement is a common feature of sarcoidosis and mucosal abnormalities may be evident in the respiratory tract. However, firm data establishing the clinical features and prognosis of sarcoidosis in these patients is lacking although the incidence of endobronchial disease is high. The purpose of this study was to evaluate the clinical features of the patients with limited, diffuse and no endobronchial involvement. Another aim was to investigate the prognostic differences between these patients. Methods: We conducted a retrospective study to evaluate the clinical and laboratory findings of 48 patients with endobronchial sarcoidosis and 50 patients without endobronchial involvement seen at our institution. The patients fulfilled the clinical, radiologic or both features of sarcoidosis supported by the histopathologic evidence of noncaseiting granulomas. Six to ten bronchial biopsies were taken from each patient. The sample was considered positive if it demonstrated noncaseiting granulomas with negative fungal and mycobacterial cultures. The patients were classified into three groups according to the histopathologic biopsy results: 1) No endobronchial involvement, 2) Limited endobronchial involvement: One biopsy site positive and 3) Diffuse endobronchial involvement: Two or more biopsy sites positive for noncaseaiting granulomas. Results: Bronchial biopsy was positive in 82% of the abnormal appearing airways while it was diagnostic in 36% of the normal appearing mucosa. The most frequent bronchoscopic appearence was miliary infiltration. Nodular, erythematous lesions and edematous mucosal swelling were other bronchoscopic findings. There were no significant differences between the three groups for FEV1, FVC, TLC, DLCO/VA serum and 24 h urinary calcium levels. Serum ACE levels were significantly higher (p<0.001) in patients with limited and diffuse bronchial involvement compared to patients with no endobronchial disease. The extrapulmonary organ involvement (p<0.001) and progressive disease incidence was more frequent (p<0.001) in patients with limited and diffuse endobronchial disease. Conclusions: Endobronchial involvement in sarcoidosis appears to be a significant predictive risk factor for progressive disease. Patients with limited or diffuse endobronchial disease have more severe extrapulmonary organ involvement and a worse prognosis than patients without endobronchial disease. Bronchoscopy may identify such patients carrying a risk factor for progressive sarcoidosis.
ABSTRACT
Aims: Endobronchial involvement may occur in patients with sarcoidosis. Although the prevalence of bronchial abnormalities is high, there are no firm data establishing the clinical features and prognosis of sarcoidosis in these patients. The aim of our study was to define the clinical characteristics and prognosis of patients with endobronchial sarcoidosis. Methods: Clinical and laboratory findings of 44 patients with endobronchial sarcoidosis and 46 patients without endobronchial involvement seen at our institution, were evaluated retrospectively. The patients fulfilled clinical, radiologic or both features of sarcoidosis supported by the histopathologic evidence of noncaseating granulomas. Six to ten bronchial biopsies were taken from each patient. The sample was considered positive if it demonstrated noncaseiting granulomas with negative bacterial, fungal and mycobacterial cultures. Results: Bronchial biopsy was more positive in 84% of the abnormal appearing airways, biopsy provided diagnostic tissue in 32% of the normal appearing mucosa. The most frequent bronchoscopic finding was miliary infiltration followed by nodular and erythematous lesions. Serum ACE, serum and urinary Ca levels were higher (51.4±14.3 IU/L vs 37.3±15.1 IU/L, p<0.01; 8.42±3.6 mg/dL vs 10.8±2.9 mg/dL, p<0.01; 244.9±32.4 mg/day vs 379.6±36.8 mg/day, p<0.01) in patients with endobronchial involvement. There was no significant difference between FEV1, FVC, TLC and DLCO/VA. The extrapulmonary organ involvement (p<0.02) and progressive disease (p<0.03) was more frequent in patients with endobronchial disease. Conclusion: Endobronchial involvement in sarcoidosis appears to be a significant predictive risk factor for progressive disease. Extrapulmonary organ involvement was also higher in these patients contributing to a worse prognosis.