Extrapulmonary sarcoidosis
Paolo Spagnolo, Vasileios Kouranos, Victoria Singh‐Curry, Thomas El Jammal, Misha Rosenbach
Abstract
Sarcoidosis is a chronic disease of unknown origin that develops when a genetically susceptible host is exposed to an antigen, leading to an exuberant immune response characterized by granulomatous inflammation. Although lung involvement is almost universal as well as the leading cause of morbidity and mortality, virtually any organ can be affected. In particular, sarcoidosis of the heart, nervous system, and eyes can be devastating, leading to death, debilitation and blindness, and a multidisciplinary approach involving expert specialists is required for prompt diagnosis and appropriate treatment. Sarcoidosis of the skin can be disfiguring, thus posing a substantial psychologic and social impact on the patients. The diagnosis is often straightforward in the presence of compatible clinical manifestations in patients with biopsy-proven sarcoidosis, but is challenging when extrapulmonary signs/symptoms occur in isolation. Corticosteroids remain the first line therapy, with immunosuppressive or biologic agents being reserved to patients failing or experiencing side effects from steroids or developing refractory disease. • Extrapulmonary manifestations are a major cause of morbidity and mortality in sarcoidosis. • Sarcoidosis of the heart, nervous system, and eyes can be catastrophic; skin lesions can affect psychological functioning. • The diagnosis may be straightforward, but is challenging when extrapulmonary disease occurs in isolation. • A multidisciplinary approach involving expert specialists is instrumental to prompt diagnosis and appropriate management.