Unilateral Endoluminal Stenosis Secondary to Pulmonary Sarcoidosis

Abstract

INTRODUCTION: Endoluminal stenosis of proximal bronchi (ESPB) is a rare and severe manifestation of pulmonary sarcoidosis. CASE PRESENTATION: A 42 year old African American gentleman with history of night sweats and wheezing presented to the hospital with sudden onset chest pain due to right sided pneumothorax. CT chest revealed hemorrhagic bullae occupying most of the right middle lobe (RML) with partial collapse of the right upper lobe (RUL) and mediastinal lymphadenopathy. Mediastinoscopy was performed with right middle lobectomy followed by flexible bronchoscopy which revealed 90% stenosis of RUL bronchus, 70% stenosis of the bronchus intermedius (BI) and normal left bronchial tree. Pathology revealed necrotic tissue from RML and non-caseating granulomas from a station 2R lymph node. After an extensive negative workup to rule out other etiologies, the patient was diagnosed with pulmonary sarcoidosis. He was started on 50 mg of prednisone daily followed 6 months later with methotrexate 15 mg weekly. A repeat airway exam at 6 months showed continued stenosis of the RUL bronchus and BI. He was therefore sent for balloon dilation of his BI. Eight months later CT chest revealed partial re-expansion of the RUL collapse. DISCUSSION: ESPB is defined as bronchoscopic narrowing of at least 50% of the bronchial lumen (1). Although an obstructive ventilatory deficit is described in up to 63% of patients with pulmonary sarcoidosis, the prevalence of ESPB was reported in 0.72% of 2500 patients in a study conducted by Chambellan el al in 2005, highlighting how rare this manifestation of disease is. Of the patients with multiple stenosis in this study, only one patient had significant narrowing of the BI (0.6% of all patients). Early treatment within three months of diagnosis correlated with better outcomes, with corticosteroids remaining the first line treatment. Endobronchial airway procedures have been described for patients with incomplete response to medical therapy. Lung transplantation remains a definitive option in selected cases of refractory and late stage disease. CONCLUSIONS: Early diagnosis and treatment of ESPB carries the best prognosis; however refractory disease is challenging and may require advanced airway procedures for treatment. Pulmonologists should have a heightened awareness of potential treatment challenges including airway intervention to improve outcomes for patients.