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Löfgren's Syndrome

Human Leukocyte Antigen Strongly Influences the Disease Course

¹ Department of Medicine, Division of Respiratory Medicine, Karolinska University Hospital Solna, Stockholm, Sweden

Correspondence and requests for reprints should be addressed to Dr. Johan Grunewald, M.D., Ph.D., Department of Medicine, Division of Respiratory Medicine, Lung Research Laboratory L4:01, Karolinska Hospital, S-171 76 Stockholm, Sweden. E-mail: johan.grunewald{at}ki.se

Rationale: Sarcoidosis may consist of a number of distinct disease entities, one of which could be Löfgren's syndrome. Patients with Löfgren's syndrome have an acute onset of erythema nodosum (EN) and/or periarticular 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 Löfgren's syndrome.

Objectives: To investigate whether human leukocyte antigen type influences clinical manifestations, including the disease course in Löfgren's syndrome.

Methods: We clinically characterized and HLA-DRB1 typed 301 patients with Löfgren's syndrome. A total of 275 of the patients were followed for more than 2 years and classified as having a nonresolving 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 Löfgren'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 Löfgren's syndrome, we can search for additional markers of importance for developing a resolving or a nonresolving disease, respectively.

Key Words: Löfgren's syndrome • sarcoidosis • HLA

AT A GLANCE COMMENTARY Scientific Knowledge on the Subject Löfgren's syndrome is a distinct form of sarcoidosis, which may constitute a disease entity by its own. Patients with Löfgren's syndrome are considered to have a good prognosis. What This Study Adds to the Field This study shows a strong association in Löfgren's syndrome between clinical manifestations and a single genetic (HLA) variant (HLA-DRB1*03).

 

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