Löfgren’s Syndrome

Abstract

Löfgren’s syndrome presents as acute sarcoid arthritis, with a triad of hilar adenopathy, acute polyarthritis and erythema nodosum. Löfgren’s syndrome is self-limited, erythema nodosum, hilar adenopathy and acute polyarthritis usually resolve within a few weeks to months, however polyarthritis can last for up to 2 years. Treatment involves symptomatic control with NSAIDs/colchicine or oral glucocorticoids until symptoms resolve, if disease is resistant to these therapies, hydroxychloroquine, methotrexate or infliximab can be used. Löfgren’s syndrome is a rare presentation of sarcoidosis occurring in only about 5–10% of sarcoid patients. It is, however, important to recognize as it is the most common form of acute sarcoid arthritis and prompt treatment can prevent unnecessary prolonged discomfort for patients.

Löfgren Syndrome: A Case Report and Treatment Challenge

“…Conversely, LöS represents an exception, as it is typically diagnosed based on its characteristic clinical profile, with a specificity of 95%, thereby obviating the necessity for histopathological examination and other additional tests [ 5 , 6 , 8 ]. Nonetheless, certain diseases must still be systematically excluded (TB, lymphoma, reactive arthritis, enteropathies, systemic vasculitis, and lupus).…”

“…It is considered an idiopathic disorder, and extensive research suggests that it arises from an interplay between genetic susceptibility and environmental exposures (infections in particular). This interaction triggers an antigen presentation reaction by macrophages to T helper lymphocytes (mainly CD4), leading to an inflammatory and immune response, culminating in the formation of non-caseating granulomas - the distinctive hallmark of sarcoidosis, although not pathognomonic [ 1 , 4 , 5 ].…”

“…When accompanied by bilateral hilar lymphadenopathy and symmetrical inflammatory arthralgias or arthritis, it usually represents an acute form of sarcoidosis known as Löfgren syndrome (LöS) [ 7 , 8 ]. LöS constitutes 5-10% of sarcoidosis cases, yet it is indeed a distinct phenotype of the disease [ 5 , 7 ]. Unlike sarcoidosis, which typically has a more gradual onset and often progresses to chronicity, LöS develops acutely and tends to be self-limited.…”

“…Unlike sarcoidosis, which typically has a more gradual onset and often progresses to chronicity, LöS develops acutely and tends to be self-limited. It is more common in women, while men are more likely to present with arthritis only [ 3 , 5 , 9 ]. Constitutional symptoms, such as fever, malaise, and fatigue, may also be present [ 1 , 5 ].…”

“…It is more common in women, while men are more likely to present with arthritis only [ 3 , 5 , 9 ]. Constitutional symptoms, such as fever, malaise, and fatigue, may also be present [ 1 , 5 ].…”