Lofgren's Syndrome Human Leukocyte Antigen Strongly Influences the Disease Course

Rationale Sarcoidosis may consist of a number of distinct disease entities, one of which could be Lofgren's syndrome. Patients with Lofgren's syndrome have an acute onset of erythema nodosum (EN) and/or perarticular inflammation or arthritis of the ankles, with bilateral hilar lymphadenopathy (and in some cases parenchymal Infiltrates) and usually fever. There is a known association between HLA-DRB1*03 and Lofgren's syndrome. Objectives: To Investigate whether human leukocyte antigen type Influences clinical manifestations, Including the disease course In Lofgren's syndrome. Methods: We clinically characterized and HLA-DRB1 typed 301 patients with Lofgren's syndrome. A total of 275 of the patients were followed for more than 2 years and classified as having a non-resolving or a resolving disease. Measurements and Main Results: Almost every DRB1*03-positive patient had a resolving disease within 2 years, and 49% of the DRB1*03-negative patients developed a nonresolving disease. Mucosal granulomas were identified significantly more often in DRB1*03-negative patients. Among DRB1*03-negative patients who were treated with oral steroids at disease onset, 80% developed a nonresolving disease. Conclusions: Patients with Lofgren's syndrome have a different disease course depending on whether they are DRB1*03 positive or not. This observation has clinical implications, and by comparing DRB1*03-positive and DRB1*03-negative patients with Lofgren's syndrome, we can search for additional markers of importance for developing a resolving or a nonresolving disease, respectively.