Pulmonary fibrosis in sarcoidosis. Clinical features and outcomes

Abstract

Sarcoidosis is a systemic inflammatory disease with a predilection for the respiratory system. Although most patients enter remission and have good long-term outcomes, up to 20% develop fibrotic lung disease, whereby granulomatous inflammation evolves to pulmonary fibrosis. There are several radiographic patterns of pulmonary fibrosis in sarcoidosis; bronchial distortion is common, and other patterns, including honeycombing, are variably observed. The development of pulmonary fibrosis is associated with significant morbidity and can be fatal. Dyspnea, cough, and hypoxemia are frequent clinical manifestations. Pulmonary function testing often demonstrates restriction from parenchymal involvement, although airflow obstruction from airway-centric fibrosis is also recognized. Complications of fibrotic pulmonary sarcoidosis include pulmonary hypertension from capillary obliteration and chronic aspergillus disease, with hemoptysis a common and potentially life-threatening manifestation. Immunosuppression is not always indicated in end-stage sarcoidosis. Lung transplantation should be considered for patients with severe fibrotic pulmonary sarcoidosis, as mortality is high in these patients.