Sarcoidosis presenting with and without Löfgren's syndrome: Clinical, radiological and behavioral differences observed in a group of 691patients

Abstract

Objectives: Just a few series of Löfgren's syndrome have been reported. Our aim was to describe the epidemiology and clinical profile of sarcoidosis patients presenting with Löfgren's syndrome vs. non-Löfgren's syndrome.

Methods: Retrospective cohort study of 691consecutive patients with sarcoidosis diagnosed at the Bellvitge University Hospital in Barcelona, Spain, between 1976 and 2018.

Results: Three hundred and nine patients (44.7%) were diagnosed with Löfgren's syndrome and 382with non-Löfgren's syndrome (55.3%). The mean age at diagnosis was 39.8years-old (SD 11.7) vs. 46.6 (SD 14.5) (P<0.001). 249 patients (80.6%) vs. 218 (57.1%) were female (P<0.001), and mostly Caucasians (304, 98.4% vs. 351, 91.9%, P=0.002). Out of the total 309, Löfgren's syndrome patients developed more frequently fever and articular involvement, and 45 (14.6%) presented with isolated periarticular ankle inflammation. When compared, radiological stages at diagnosis were more advanced in non-Löfgren's syndrome patients: stage 0 (2.9% vs. 14.7%), stage I (82.5% vs. 41.4%), stage II (14.6% vs. 29.3%), and stage III/IV (0 vs. 14.7%) (P<0.001). Chronic trend>2years was more prevalent in non-Löfgren's syndrome (66, 22.6% vs. 233, 67.4%; P<0.001), as well as the proportion of patients in whom treatment was needed (58, 18.8% vs. 224, 58.6%; P<0.001). Risk factors related to chronic trend>2 years were older age, stage II at diagnosis and the need of treatment.

Conclusions: Löfgren's syndrome is a well-differentiated form of sarcoidosis with persuasive different epidemiological, clinical, radiological and prognostic features.

Keywords: Erythema nodosum; Löfgren's syndrome; Sarcoidosis.