Aim: Endoluminal stenosis of proximal bronchi (ESPB) is a potentially severe manifestation of sarcoidosis. Unusual clinical presentation and variable response to medical treatment require specific attention to diagnosis and follow-up.
Design: Of 2,500 patients with sarcoidosis seen at our institution, we retrospectively identified 18 patients with stage 1-3 sarcoidosis and ESPB. Clinical manifestations, endoscopic findings, pulmonary function tests, follow-up, and therapeutic response were assessed.
Results: Respiratory symptoms were present in 17 patients (94%): cough and dyspnea (89% each), wheezing (83%), and hemoptysis (11%). Generalized symptoms (67%) and extrapulmonary manifestations (72%) of sarcoidosis were frequent. Three bronchoscopic patterns were observed: single stenosis (n = 3), multiple stenoses (n = 12), or diffuse narrowing of the bronchial tree (n = 3). The two former groups accounted for 45 ESPBs located in the left upper lobe (44.5%), the right upper and middle lobes (15.5% each), and the left lower lobe (11%). ESPBs were due to mural thickening of bronchi (n = 16) or associated with extrinsic compression by lymphadenopathy (n = 2). Endobronchial biopsies uniformly confirmed the presence of granulomas. FEV(1)/FVC ratio was < 70% in 12 patients (66.7%), with a correlation between the decrease of FEV(1)/FVC ratio and the number of ESPBs (R(2) = 0.31; p = 0.02). Patients treated with oral corticosteroids (n = 12) or methotrexate (n = 1) within the first 3 months had a good prognosis, whereas patients in whom treatment was delayed by > 3 months (n = 4) or who did not receive any systemic treatment (n = 1) acquired fixed ESPB and persistent ventilatory defects.
Conclusions: ESPB is a rare and serious complication of sarcoidosis. Its clinical hallmarks include multiple respiratory symptoms, multiorgan involvement, and generalized symptoms. Treatment has to be started early to avoid the development of fixed stenotic lesions and irreversible pulmonary function impairment.