Löfgren Syndrome – Symptoms, Diagnosis and Treatment
Löfgren syndrome is an acute form of sarcoidosis characterized by fever, joint swelling, erythema nodosum, and enlarged lymph nodes in the chest.
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Löfgren syndrome is an acute form of sarcoidosis characterized by fever, joint swelling, erythema nodosum, and enlarged lymph nodes in the chest.
What is Löfgren Syndrome?
Löfgren syndrome is an acute and typically self-limiting form of sarcoidosis, an inflammatory disease in which small clusters of immune cells – known as granulomas – form in various tissues. It was first described in 1953 by Swedish physician Sven Löfgren. The syndrome most commonly affects younger adults and is recognized by a characteristic combination of symptoms that appear together.
Causes
The exact cause of Löfgren syndrome is not yet fully understood. It is considered an immune-mediated condition in which the immune system overreacts and forms granulomas. Several contributing factors have been identified:
- Genetic predisposition (specific HLA genotypes, particularly HLA-DRB1*03)
- Infectious triggers, such as bacteria like Mycobacterium tuberculosis or Propionibacterium acnes
- Environmental factors and occupational exposure to certain dusts or chemicals
Symptoms
Löfgren syndrome is defined by a classic triad of three hallmark findings:
- Erythema nodosum: Painful, reddish nodules beneath the skin, typically appearing on the lower legs
- Bilateral hilar lymphadenopathy: Enlargement of lymph nodes on both sides of the lung roots, visible on chest X-ray
- Acute arthritis: Joint pain and swelling, most commonly affecting the ankles, knees, and wrists
Additional common symptoms include:
- Fever
- General malaise and fatigue
- Night sweats
- Cough or mild respiratory discomfort
Diagnosis
The diagnosis of Löfgren syndrome is primarily clinical, meaning it is based on the characteristic pattern of symptoms. Invasive procedures such as tissue biopsy are generally not required when the classic symptom combination is present. The following diagnostic tools are commonly used:
- Chest X-ray: To confirm bilateral hilar lymphadenopathy
- Blood tests: Elevated inflammatory markers (CRP, ESR), elevated angiotensin-converting enzyme (ACE), possible hypercalcemia
- CT scan of the chest: For a more detailed assessment of lymph nodes and lung tissue in unclear cases
- Skin biopsy: For histological confirmation of erythema nodosum if needed
- Exclusion of other conditions: Such as tuberculosis, lymphoma, or other causes of lymph node enlargement
Treatment
In most patients, Löfgren syndrome follows a self-limiting course, resolving spontaneously within 6 to 24 months without specific treatment. The overall prognosis is excellent. Treatment focuses on relieving symptoms:
Symptomatic Treatment
- Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen or naproxen: To relieve joint inflammation and fever
- Rest and leg elevation: Recommended when erythema nodosum is pronounced
- Compression stockings: To reduce leg swelling
Corticosteroids
In severe cases or when NSAIDs are insufficient, corticosteroids such as prednisolone may be prescribed. These medications have a strong anti-inflammatory effect and can accelerate symptom resolution.
Follow-up Care
Regular medical check-ups are important to ensure complete resolution of the disease and to detect any transition to chronic sarcoidosis. A small proportion of patients may go on to develop a chronic form of the condition.
Prognosis
Löfgren syndrome carries a very favorable prognosis compared to other forms of sarcoidosis. Spontaneous remission rates exceed 80%. Patients carrying the HLA-DRB1*03 genotype are particularly likely to experience a benign course.
References
- Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. New England Journal of Medicine. 2007;357(21):2153-2165.
- Mana J, Gomez-Vaquero C, Montero A, et al. Löfgren's syndrome revisited: a study of 186 patients. American Journal of Medicine. 1999;107(3):240-245.
- Spagnolo P, Rossi G, Trisolini R, et al. Pulmonary sarcoidosis. Lancet Respiratory Medicine. 2018;6(5):389-402.
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