Sarcoidosis: an update for the primary care physician

Sarcoidosis is a systemic inflammatory disorder of unknown etiology, characterized by the formation of noncaseating granulomas, multisystem involvement, intrathoracic (pulmonary and lymph node) involvement in more than 90% of cases, and activation of T cells and macrophages at sites of granulomatous inflammation with the release of various chemokines and cytokines including tumor necrosis factor (TNF)-alpha. Despite the fact that sarcoidosis is seen all over the world, the study of its epidemiology has been a challenge for several reasons, including the lack of consistency in case definition, variability in disease presentation, the lack of sensitive and specific diagnostic tests, underrecognition and misdiagnosis of the disease, and a dearth of systematic epidemiologic studies. The ACCESS (A Case Control Etiologic Study of Sarcoidosis) study, a multicenter study done at 10 centers in the United States, is an attempt to better characterize the disease. A study of a subset of patients in the ACCESS study showed there was a delay in making the diagnosis of sarcoidosis, even when patients presented with pulmonary symptoms. The role of the primary care physician includes the establishment of a supportive physician-patient relationship. The morbidity and psychosocial problems associated with this condition necessitate educating patients about the disease because of the overwhelming concerns about its etiology, prognosis, and potential adverse effects of medication. This supportive role enhances patients' roles in the process of shared decision making during testing and treatment decisions.