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AB0919 A Look at LÖFgren Syndrome: 80 Cases in Two Universitary Hospitals
  1. B. Segura1,
  2. S. Holgado1,
  3. L. Mateo-Soria1,
  4. M. Martínez-Morillo1,
  5. A. Riveros-Frutos1,
  6. J.M. Pego-Reigosa2,
  7. M. Carnicero-Iglesias2,
  8. S. Rodriguez-Muguruza1,
  9. X. Tena-Marsà1,
  10. A. Olivé-Marqués1
  1. 1Rheumatology Service, Universitary Hospital Germans Trias i Pujol, Badalona, Barcelona
  2. 2Rheumatology Section, Universitary Hospital of Vigo, Vigo, Galicia, Spain

Abstract

Background Löfgren's syndrome is a common form of sarcoidosis in the Mediterranean region. Characterized by bilateral hilar lymphadenopathy, erythema nodosum and arthritis,usually symmetrical and predominate in lower extremities.

Objectives To describe the clinical characteristics, treatment and outcome of a series of patients diagnosed with Löfgren syndrome.

Methods Retrospective design (1984–2013) multicenter (2 university hospitals) with a total catchment area of 1,015,000 inhabitants. Löfgren syndrome was defined as the association of erythema nodosum and/or ankle arthritis and bilateral hilar lymphadenopathy in chest radiography. Exclusion criteria:presence of some other granulomatous disease.

Results 80 patients were diagnosed: 29 men and 51 women (mean age: 42.3). The mean follow-up was 54 months. 48 (60%) had the classic triad of the disease; 18 (22.5%) hilar adenopathy and arthritis,13 (16.7%) hilar adenopathy and erythema nodosum. In one case the diagnosis was casual. Ocular manifestations occurred in 11 patients with the most common xerophthalmia. 28 patients had fever, 18 fatigue and 4 significant weight loss. All had abnormalities in the study of the chest and were classified in the stage I-II. Pulmonary function tests were perfomed in 55 patients,was pathological in 6. In laboratory tests it was seen: normocytic normochromic anemia in 26 patients (32.5%), lymphopenia in 6 (7.5%), bicytopenia in 4 (5%), hypercalcemia in 4 and hypercalciuria in 1.T he ESR was determined in 78 patients with a mean of 52.4±37.2 mm/hour. The tuberculin test was positive in 6 cases. In these patients, active tuberculosis disease is excluded. 39 biopsies were performed: 23 transbronchial, 11 skin, 3 lymph node, 2 muscular and 1 of lower lid. Were available for pathologic confirmation of sarcoidosis in 28 cases (35%). It was nonspecific in 11 patients. NSAID treatment was performed in 54 patients and glucocorticoids in 33. The remission in the first 2 years after diagnosis was observed in 87.5% of cases. 14 patients relapsed: 7 performed glucocorticoids, 3 NSAIDs and 4 had not received treatment.

Table 1 shows the main characteristics of the patients compared to the most important series in the literature.

Table 1

Conclusions Löfgren's syndrome is a common clinical form of sarcoidosis in the Mediterranean area. Should be suspected in the presence of erythema nodosum or periarticular swelling ankles. The clinical and radiological pattern can obviate biopsy. The prognosis is good and recurrence scarce.

Disclosure of Interest None declared

DOI 10.1136/annrheumdis-2014-eular.2955

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