Journal
SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE
Volume 41, Issue 5, Pages 733-740Publisher
THIEME MEDICAL PUBL INC
DOI: 10.1055/s-0040-1710371
Keywords
sarcoidosis; prognostic factors; nonthreatening course; Lofgren's syndrome; bilateral hilar lymphadenopathy; erythema nodosum
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Sarcoidosis is a systemic granulomatous disease of unknown etiology. The outcome is quite variable and is mainly related to persistent inflammatory processes and the development of fibrosis. Many prognostic factors have been described, but the disease evolution is not yet entirely known. The nonthreatening course is characterized by spontaneous involution or stability after treatment withdrawal. Lofgren's syndrome is a subset within the spectrum of sarcoidosis phenotypes, composed of acute onset of fever, bilateral hilar lymphadenopathy, erythema nodosum and/or bilateral ankle periarticular inflammation/arthritis, specifically characterized by a self-limiting disease course. In contrast, advanced fibrotic sarcoidosis with pulmonary hypertension phenotype is correlated with a poor prognosis. Further studies are necessary to detail phenotypes to better understand the mechanisms of the disease and plan future clinical therapeutic studies.
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