Löfgren syndrome
Acute sarcoidosis with triad of erythema nodosum, hilar lymphadenopathy, arthritis.
Löfgren syndrome is an acute form of sarcoidosis, an inflammatory disease. Its hallmarks include swollen chest lymph nodes, tender red bumps on the shins, fever, and joint pain. First identified in 1953 by Swedish physician Sven Halvar Löfgren, the syndrome shows a higher prevalence in women and in people of Scandinavian, Irish, African, and Puerto Rican descent. Some experts have noted that the condition lacks precise definition.
The classic signs are hilar lymphadenopathy—enlarged lymph nodes near the inner lungs visible on X-ray—and erythema nodosum, the tender red nodules on the shins, which appear mainly in women. Arthritis, more common in men, often strikes the lower extremities, especially the ankles, and can be acute. Fever may also occur. The diagnostic triad consists of erythema nodosum, bilateral hilar lymphadenopathy, and joint pain.
Genetically, the HLA-DRB1*03 allele is strongly linked to Löfgren syndrome.
When all three triad features are present, the diagnosis is highly specific—over 95%—and no further imaging or lab tests are needed.
First-line treatment usually involves nonsteroidal anti-inflammatory drugs (NSAIDs). Alternatives include colchicine or low-dose prednisone. In 90% of cases, symptoms resolve within six weeks of starting NSAIDs or corticosteroids. Rarely, chronic sarcoid arthropathy leads to long-term joint damage, which may require disease-modifying antirheumatic drugs (DMARDs).
The outlook is favorable: over 90% of patients recover within two years. By contrast, those with lupus pernio, cardiac involvement, or neurologic disease rarely see remission.
- described_by
- Sven Halvar Löfgren
- year_described
- 1953
- field
- Medicine
- nationality
- Swedish
- known_for
- Describing Löfgren syndrome, a form of acute sarcoidosis
Lore & Background
Löfgren syndrome is characterized by enlargement of the lymph nodes near the inner border of the lungs (hilar lymphadenopathy) as seen on x-ray, and tender red nodules (erythema nodosum) classically present on the shins, predominantly in women. It may also be accompanied by arthritis, which is more prominent in men, and fever. The arthritis is often acute and involves the lower extremities, particularly the ankles. The condition consists of the triad of erythema nodosum, bilateral hilar lymphadenopathy on chest radiograph, and joint pain.
Recent studies have demonstrated that the HLA-DRB1*03 is strongly associated with Löfgren syndrome. The triad of erythema nodosum, acute arthritis, and bilateral hilar lymphadenopathy is highly specific (>95%) for the diagnosis. When the triad is present, further testing with additional imaging and laboratory testing is unnecessary.
Treatment with NSAIDs (nonsteroidal anti-inflammatory drugs) is usual; colchicine or low-dose prednisone may also be used. Treatment with NSAIDs or corticosteroids leads to symptomatic relief 90% of the time within six weeks of symptom onset. Rarely, long-term joint damage (chronic sarcoid arthropathy) may develop, and disease modifying antirheumatic drugs (DMARDs) are used for such cases.
Reader's Guide
Löfgren syndrome is significant as a distinct, acute presentation of sarcoidosis with a characteristic triad that allows for highly specific diagnosis without extensive testing. Its strong association with HLA-DRB1*03 highlights a genetic component, and its good prognosis—with over 90% of patients experiencing disease resolution within two years—contrasts sharply with other forms of sarcoidosis such as lupus pernio or those with cardiac or neurologic involvement, which rarely remit. The condition's description by Sven Halvar Löfgren in 1953 established a clinically useful entity, though some have considered it imprecisely defined. Its higher prevalence in women and certain ethnic groups (Scandinavian, Irish, African, Puerto Rican) informs epidemiological understanding. Treatment is typically symptomatic with NSAIDs or corticosteroids, achieving relief in most cases within weeks, while chronic arthropathy is rare and managed with DMARDs. The syndrome's legacy lies in its clear diagnostic criteria and favorable outcome, aiding in the management and prognosis of acute sarcoidosis.
Did You Know?
- Löfgren syndrome is more common in women than men.
- The triad of erythema nodosum, acute arthritis, and bilateral hilar lymphadenopathy is >95% specific for diagnosis.
- Treatment with NSAIDs or corticosteroids leads to symptomatic relief 90% of the time within six weeks.
- Over 90% of patients experience disease resolution within two years.
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