Pulmonary Fibrosis in Sarcoidosis
Joel Francesqui 1, Sanjay Kalra 2, Eva M Carmona 2
1 Servei de Pneumologia, Interstitial Lung Disease Program, Respiratory Institute, Hospital Clínic, Barcelona, Spain; 2 Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota, USA
*Correspondence: Eva M Carmona, Email not available
Abstract
Sarcoidosis is a systemic granulomatous disease with an intrathoracic involvement in about 90% of the cases. Self-limited disease is the most common presentation; however, 10-20% of these patients may progress to fibrotic forms, so-called advanced pulmonary sarcoidosis or end-stage sarcoidosis. Recognition of this phenotype is important as it carries a poor prognosis and a limited response to treatment. Clinical manifestations of fibrotic sarcoidosis include common symptoms like dyspnea and chronic cough. Radiologically, typical fibrotic patterns include bronchial distortion, honeycomb and diffuse linear fibrosis. Unlike non-fibrotic sarcoidosis, aspergillus, bronchiectasis- related bacterial infections, venous thromboembolic disease and pulmonary hypertension are complications most commonly associated with this fibrotic stage. Resistance to usual treatments is a known feature, but recent evidence shows that antifibrotic agents such as Nintedanib may have a role to slow pulmonary function deterioration. In the absence of active disease, referral for lung transplant evaluation is recommended.
