Sarcoidosis presenting with and without Löfgren's syndrome: Clinical, radiological and behavioral differences observed in a group of 691 patients - 29/10/19

Doi : 10.1016/j.jbspin.2019.10.001

Manuel Rubio-Rivas ^a,⁎ , Jonathan Franco ^b, Xavier Corbella ^a,^c,^d
^a Autoimmune Diseases Unit, Department of Internal Medicine, Bellvitge University Hospital, Bellvitge Biomedical Research Institute-IDIBELL, University of Barcelona, Barcelona, Spain
^b Department of Internal Medicine, Dexeus University Hospital, Barcelona, Spain
^c Faculty of Medicine and Health Sciences, Universitat Internacional de Catalunya, Barcelona, Spain
^d Group of Evaluation of Health Determinants and Health Policies, Hestia Chair in Integrated Health and Social Care, Barcelona, Spain

^⁎Corresponding author at: Bellvitge University Hospital, Hospitalet de Llobregat, Barcelona, 08907, Spain.Bellvitge University Hospital, Hospitalet de LlobregatBarcelona08907Spain

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Highlights

•	Largest Löfgren's cohort reported to date.
•	They are mostly younger, females and frequently accompanied by fever and articular involvement.
•	Older age, stage II at diagnosis and the need of treatment are related to chronic trend.
•	Whether Löfgren's syndrome represents a subset or a separate entity in sarcoidosis is yet to be clarified.

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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.

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Keywords : Sarcoidosis, Löfgren's syndrome, Erythema nodosum