Course of chronic hilar sarcoidosis in relation to markers of granulomatous activity

Studies of the course of sarcoidosis have emphasized that patients with hilar or mediastinal adenopathy usually recover within several years or develop dissemination to the lungs. Chronic hilar and mediastinal adenopathy persisting with little or no change for many decades is an important subgroup that has not received adequate attention. Twelve such patients have been studied. Seven remained asymptomatic, despite persistent adenopathy, for a mean period of 16 years; two with disfiguring facial sarcoids received corticosteroids for 18 and 27 years, respectively, and three patients after ten years of stable adenopathy developed pulmonary infiltrates. Tests performed on patients with hilar adenopathy to evaluate cellular activity after a mean interval of over 16 years included Kveim reaction (positive in nine of ten), serum angiotensin converting enzyme (elevated in eight of 12), and gallium-67 scanning (hilar uptake in all eight tested). Results were similar for patients who remained well and for those who had symptomatic or progressive disease, indicating that these parameters of granulomatous activity do not reflect the duration of the disease, its outcome, or the need for treatment.