Initial Treatment for Löfgren's Syndrome
For most patients with Löfgren's syndrome, initial treatment consists of NSAIDs alone, as this acute variant of sarcoidosis is typically self-limiting with an excellent prognosis. 1
Clinical Context and Recognition
Löfgren's syndrome is a highly distinctive acute presentation of sarcoidosis that is considered "highly supportive" of the diagnosis and often does not require tissue biopsy for confirmation. 1 The classic triad includes:
- Bilateral hilar lymphadenopathy 1, 2
- Erythema nodosum (often presenting as bilateral lower extremity erythema and swelling) 2, 3, 4
- Acute arthritis or periarthritis (most commonly involving the ankles) 2, 3, 4
- Fever is frequently present 5, 3
First-Line Treatment Approach
NSAIDs as Primary Therapy
NSAIDs should be the initial treatment for symptomatic relief in Löfgren's syndrome. 2 This approach is justified because:
- The syndrome is self-limiting in the vast majority of cases 2, 6
- NSAID therapy leads to rapid clinical improvement 2
- The prognosis is excellent without aggressive immunosuppression 1
When to Consider Corticosteroids
Systemic corticosteroids should be reserved for specific situations:
- Severe or refractory symptoms that do not respond adequately to NSAIDs 7
- Symptomatic sarcoidosis with significant organ involvement beyond the typical Löfgren's presentation 7
- Prednisone is FDA-approved for "symptomatic sarcoidosis" 7
If corticosteroids are needed, use moderate doses (0.5-1.0 mg/kg prednisone equivalent) rather than high-dose therapy. 1 This is substantially lower than the high-dose regimens (up to 16 weeks) used for other forms of sarcoidosis or glomerular diseases. 1
Critical Pitfalls to Avoid
Misdiagnosis as Cellulitis
Löfgren's syndrome frequently masquerades as bilateral lower extremity cellulitis. 2 Key distinguishing features:
- Lack of response to antibiotics should prompt reconsideration 2, 5
- Bilateral presentation is atypical for cellulitis 2
- Accompanying systemic symptoms (fever, polyarthritis) suggest systemic disease 2, 3
- Early thoracic imaging can prevent misdiagnosis and unnecessary antibiotic treatment 2
Overtreatment with Immunosuppression
Unlike chronic sarcoidosis or other inflammatory conditions requiring prolonged immunosuppression:
- Do not initiate high-dose, prolonged corticosteroid regimens as used in FSGS or other glomerular diseases 1
- Avoid second-line immunosuppressants (azathioprine, mycophenolate, rituximab, CNIs) unless there is progression to chronic sarcoidosis with organ-threatening disease 1, 8, 9
Monitoring and Follow-Up
Close monitoring is essential because:
- Some patients may experience recurrence or relapse 3
- A small subset may progress to chronic sarcoidosis requiring more intensive therapy 3
- Secondary forms of acute sarcoidosis may require additional immunomodulatory therapies 3
The self-limiting nature of Löfgren's syndrome distinguishes it from other sarcoidosis presentations and justifies the conservative initial approach with NSAIDs rather than aggressive immunosuppression. 2, 6