Löfgren's syndrome, a benign form of sarcoidosis, is an association of erythema nodosum and bilateral hilar lymphadenopathy. Mañá and colleagues reviewed clinical features and outcomes in patients with Löfgren's syndrome.
The authors prospectively followed patients with Löfgren's syndrome who were diagnosed over a 22-year period. Löfgren's syndrome was defined as erythema nodosum, periarticular ankle inflammation, or both, plus bilateral hilar or right paratracheal adenopathy. The diagnosis of sarcoidosis was made according to accepted criteria, and sarcoid activity was similarly determined (see accompanying table). Patients without a biopsy-confirmed case of sarcoid were included if their tuberculin test was negative and other diseases were ruled out.
Information about the season of onset, radiographic results, clinical course and laboratory test results was collected on all patients diagnosed with Löfgren's syndrome. The blood work included determination of angiotensin-converting enzyme levels. Treatment during the study was not directed; most of the patients received nonsteroidal anti-inflammatory agents or potassium iodide. A total of 186 patients was included in the study. Most of the patients were white women, and the mean age was 37 (± 11) years. The onset of symptoms in nearly one half of the patients was between April and June. Almost all (93 percent) of the patients had erythema nodosum or periarticular ankle inflammation as their presenting problem; these were often accompanied by arthralgia and low-grade fever. Most of the baseline chest radiographs (81 percent) showed hilar or paratracheal adenopathy; none had parenchymal involvement at the time of presentation. One half of the patients had elevated angiotensin-converting enzyme levels, and all of the patients who had gallium-67 scans showed evidence of hilar or right paratracheal uptake. The disease had become inactive in 92 percent of the patients within two years of diagnosis (usually between three and 12 months). Only 6 percent of the patients had recurrent disease between two and 20 years after diagnosis.
The patients with increased levels of angiotensin-converting enzyme were more likely to have persistent or recurrent disease. Most (96 percent) of those with normal levels did not have persistent or recurrent disease. The authors conclude that certain tests should be performed. Specifically, patients who present with periarticular ankle inflammation should have a chest radiograph to look for hilar lymphadenopathy. Patients, whether asymptomatic or with erythema nodosum or uveitis, may have a diagnosis of sarcoidosis supported by a finding of bilateral lymphadenopathy along with an elevated angiotensin-converting enzyme level plus a negative tuberculin test. If the chest radiograph is not helpful, a typical pattern on gallium-67 scan may prove highly suggestive. Routine biopsy is not needed to confirm the diagnosis but may be needed in patients whose presentation is atypical.
Lymphoma, tuberculosis or malignancy must be excluded in the face of a chest radiograph that does not show symmetric bilateral lymphadenopathy. Patients should be reassured that Löfgren's syndrome is benign and usually self-limited. Follow-up should continue until hilar adenopathy has resolved.