Semin Respir Crit Care Med 2017; 38(04): 463-476
DOI: 10.1055/s-0037-1602380
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Löfgren's Syndrome: Diagnosis, Management, and Disease Pathogenesis

Bekir Karakaya
Interstitial Lung Diseases Center of Excellence, Department of Pulmonology, St. Antonius Hospital, Nieuwegein, The Netherlands
Both the authors contributed equally to this article.
,
Ylva Kaiser
Respiratory Medicine Unit, Department of Medicine, Solna and Center for Molecular Medicine, Karolinska Institutet and Karolinska University Hospital, Solna, Stockholm, Sweden
Both the authors contributed equally to this article.
,
Coline H. M. van Moorsel
Interstitial Lung Diseases Center of Excellence, Department of Pulmonology, St. Antonius Hospital, Nieuwegein, The Netherlands
Shared senior authorship.
,
Johan Grunewald
Respiratory Medicine Unit, Department of Medicine, Solna and Center for Molecular Medicine, Karolinska Institutet and Karolinska University Hospital, Solna, Stockholm, Sweden
Shared senior authorship.
› Author Affiliations
Further Information

Publication History

Publication Date:
27 July 2017 (online)

Abstract

Löfgren's syndrome (LS), first described in 1946 by Swedish Professor of Medicine Sven Löfgren, is a clinically distinct phenotype of sarcoidosis. Patients typically experience an acute disease onset, usually with fever, and characteristic symptoms of bilateral hilar lymphadenopathy, erythema nodosum, and/or bilateral ankle arthritis or periarticular inflammation. LS patients are well documented to have a good prognosis, which is especially true for HLA-DRB1*03+ individuals. The presence of this allele correlates closely with an accumulation of clonal CD4+ T-cell populations in the lung, suggestive of local antigen recognition. Moreover, LS differs markedly from “non-LS” sarcoidosis regarding immune cell activation, differentiation, and regulation, which may influence clinical outcome and spontaneous disease resolution.

This review offers an overview of the clinical characteristics, genetic background, and immunological characteristics of LS, as well as patient management, and reflections on future scientific challenges, emphasizing the concept of LS as a disease in its own right.