Löfgren syndrome is a specific acute clinical presentation of systemic sarcoidosis that usually manifests with lymphadenopathy, fevers, erythema nodosum, and polyarticular arthritis.
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Terminology
It is important to not confuse this syndrome with Löffler syndrome, as the names are quite similar but the conditions quite different.
Epidemiology
Löfgren syndrome has a strong predominance for females in their thirties 4.
Clinical presentation
Clinical features include:
-
constitutional symptoms
fever
malaise
-
hilar lymphadenopathy
-
arthritis
symmetrical polyarthropathy
most commonly affecting the ankles
uveitis (occasionally)
parotitis (occasionally)
Pathology
Löfgren syndrome may be associated with HLA-DRB1*03, which is also a marker of favourable prognosis 5,6.
Treatment and prognosis
Compared to run-of-the-mill sarcoidosis, patients presenting with Löfgren syndrome typically have a better overall prognosis with a self-limited clinical course (mean ~2 months 3) and spontaneous remission.
History and etymology
It was initially described by Sven Halvar Löfgren (1910-1978), a Swedish physician, in 1952 2.
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