Clinical Studies
Löfgren’s syndrome revisited: a study of 186 patients

Presented in part at the International Congress on Sarcoidosis, Granulomatous and Vasculitic Disorders, 5th World Association on Sarcoidosis and Other Granulomatous Disorders (WASOG) Meeting, Essen, Germany, September 17–19, 1997.
https://doi.org/10.1016/S0002-9343(99)00223-5Get rights and content

Abstract

PURPOSE: To evaluate the clinical features, the results of noninvasive tests and biopsies, and the outcome of patients with Löfgren’s syndrome.

SUBJECTS AND METHODS: Patients diagnosed as having Löfgren’s syndrome at a university hospital in Barcelona, Spain, from 1974 to 1996, were prospectively followed. Löfgren’s syndrome was defined as the association of erythema nodosum or periarticular ankle inflammation with unilateral or bilateral hilar or right paratracheal lymphadenopathy.

RESULTS: Löfgren’s syndrome was diagnosed in 186 patients. The mean age was 37 ± 11 years, and 157 (85%) were women. In 91 patients (49%), symptoms started during the spring (P <0.0001). Erythema nodosum, periarticular ankle inflammation, or both were present at onset in 173 patients (93%). At the time of diagnosis, 161 patients (87%) had no respiratory symptoms; 151 (81%) had stage I abnormalities on chest radiograph, 29 (16%) stage II, and 6 (3%) stage 0. Five percent of patients had decreased forced vital capacity, and 15% had decreased carbon monoxide diffusing capacity. Extrathoracic involvement was infrequent. Serum angiotensin-converting enzyme levels were increased in 50% of patients. Gallium-67 scans showed hilar uptake in all the studied patients, but it yielded useful additional diagnostic information only in those with normal chest radiographs or with unilateral hilar lymphadenopathy. The diagnosis was proven with biopsy results in 63% of patients. None of the patients without histologic confirmation were subsequently found to have a diagnosis other than sarcoidosis. In the 133 patients who were followed for a mean of almost 5 years, 11 (8%) continued to have active disease, and 8 (6%) had several recurrences between 18 months and 20 years after a complete resolution. A normal serum angiotensin-converting enzyme level at diagnosis was associated with disease resolution without recurrence.

CONCLUSION: Löfgren’s syndrome is usually a self-limiting form of sarcoidosis. Histologic confirmation is not necessary in typical cases. In a small number of patients, the disease may remain active or recur long after its onset, although usually with mild organ dysfunction.

Section snippets

Definitions and diagnostic criteria

The diagnosis of sarcoidosis was made according to the classical criteria: a compatible clinical and radiologic picture; histologic demonstration of noncaseating granulomas in one or more tissues with negative stains and cultures for mycobacteria and fungi, or a positive Kveim test; and exclusion of other granulomatous diseases. Löfgren’s syndrome was defined as the association of erythema nodosum, periarticular ankle inflammation, or both with unilateral or bilateral hilar or right

Results

The diagnosis of Löfgren’s syndrome was made in 186 patients. All were white. The mean age was 37 ± 11 years (range, 14 to 66 years) and 157 (85%) were women. In 91 patients (49%), symptoms were first noticed between April and June. This seasonal distribution was significantly greater than for the other 3-month periods (P <0.0001). In 173 patients (93%) erythema nodosum, periarticular ankle inflammation, or both, sometimes accompanied by arthralgia and low-grade fever, was the initial

Discussion

This study supports the epidemiologic data that suggest that there are differences in the presentation of sarcoidosis according to the involved population (12). All of our patients were white, with a great predominance of young women. The seasonal clustering during the spring months, which has been reported previously, suggests that an environmental factor may be important in the etiology of the disease 16, 23, 24. Interestingly, many patients initially presented with periarticular ankle

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