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Am J Respir Crit Care Med. 2009 Feb 15;179(4):307-12. doi: 10.1164/rccm.200807-1082OC. Epub 2008 Nov 7.

Löfgren's syndrome: human leukocyte antigen strongly influences the disease course.

Author information

1
Department of Medicine, Division of Respiratory Medicine, Lung Research Laboratory L4:01, Karolinska Hospital, S-171 76 Stockholm, Sweden. johan.grunewald@ki.se

Abstract

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.

PMID:
18996998
DOI:
10.1164/rccm.200807-1082OC
[Indexed for MEDLINE]

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