Cardiac Involvement in Löfgren Syndrome
Diagnosis and Evaluation
- The American Thoracic Society guidelines suggest that Löfgren syndrome is characterized by bilateral hilar lymphadenopathy, erythema nodosum, and/or periarticular arthritis, and is usually associated with a good prognosis 1, 2, 3, 4, 5, 6
- Bilateral hilar lymphadenopathy (on chest X-ray, CT, or PET) is a diagnostic criterion for Löfgren syndrome 1, 2, 3, 4, 5, 6
- Erythema nodosum and/or periarticular arthritis are also diagnostic criteria for Löfgren syndrome 1, 2, 3, 4, 5, 6
- If the classic triad is present, the diagnosis is considered "highly likely" according to the American Thoracic Society guidelines 1, 2
Cardiac Evaluation
- An abnormal EKG + cardiac symptoms (including tachycardia) combination increases the risk of cardiac sarcoidosis to 27.5% 7
- If abnormal EKG findings are present, cardiac MRI, PET, or gallium scintigraphy should be used to evaluate inflammatory activity in the heart 1, 2, 3, 4, 5, 6
- Reduced left ventricular ejection fraction without risk factors is considered a "possible" finding 1, 2, 3, 4, 5, 6
- Ventricular tachycardia without risk factors is considered a "possible" finding 1, 2, 3, 4, 5, 6
Cardiac Complications
- Atrio-ventricular block, cardiomyopathy, and treatment-responsive AV node block are considered "possible" signs of cardiac involvement 1, 2, 3, 4, 5, 6
Management Approach
- Löfgren syndrome usually has a self-limited course and excellent prognosis, but if atypical features (such as uveitis) are present, a more comprehensive evaluation is required 1, 2
Löfgren Syndrome and Uveitis Relationship
Diagnosis and Characteristics
- The American Thoracic Society guideline considers the diagnosis "highly likely" when the classic triad of Löfgren syndrome is present, and a biopsy is usually not required 8
- According to the American Thoracic Society guideline, uveitis is listed as a "possible" finding for sarcoidosis in the "physical examination" category, but it is not included in the "highly likely" characteristics of Löfgren syndrome 8
Clinical Importance
- The development of uveitis in a patient with Löfgren syndrome may suggest a deviation from the typical benign course of the disease, and may indicate more aggressive systemic sarcoidosis 8