Current best practice in the management of pulmonary and systemic sarcoidosis

Abstract

Sarcoidosis is a systemic inflammatory disease of unknown etiology that is characterized by the presence of granulomatous inflammation in affected tissues. It can affect essentially any organ system but shows a predilection for the lungs, eyes, and skin. Accurate epidemiological data are not available in the USA, but sarcoidosis is considered a ‘rare disease’ (prevalence less than 200,000). However, recent epidemiologic studies indicate that regional prevalence is much higher than previously estimated, especially among African American women. Additionally, mortality rates of patients with sarcoidosis are increasing by 3% per year over the past two decades. The most common causes of death are attributed to progressive lung disease and cardiac sarcoidosis, and the health of the patients is further compromised by other systemic manifestations. As such, the management of sarcoidosis requires a collaborative multidisciplinary approach. We aim to discuss the principles of managing sarcoidosis, including standards of care relating to pulmonary disease as well as recent advances relating to the detection and treatment of extrapulmonary manifestations.