Résumé
The macrophage activation syndrome (MAS) i a rare pediatric condition characterized by enhanced activation of the macrophage and T-cell system, with increased liberation of T-cell cytokines. Clinically it resembles a multiorgan failure syndrome of infectious etiology. There is still some debate as to the nomenclature of this syndrome, hemato-oncologists usually classify this syndrome as a histiocytic disorder, while rheumatologists define it as MAS. We review a patient in which MAS was diagnosed during the early stages of the disease. The patient had juvenile idiopathic arthritis (systemic presentation).
Sujets)
Mâle , Adolescent , Humains , Arthrite juvénile/complications , Arthrite juvénile/métabolisme , Activation des macrophages , SyndromeRésumé
Background: The diagnostic profile of patients with rheumatic diseases admitted to a general hospital is variable. Aim: To report the epidemiological profile of patients with rheumatic diseases admitted to a tertiary care hospital. Material and methods: All admissions to a Medicine ward of a general hospital and seen by the Rheumatology team were prospectively registered during one year in 1999. Patients were classified as primarily admitted for a rheumatic disease or admitted for other cause that required a consultation with the Rheumatology team. Results: One hundred forty five admissions due to rheumatic diseases were registered. Of these, 82 were due to primary rheumatic diseases. Systemic lupus erythematosus, rheumatoid arthritis and vasculitis were the main diagnoses and the mean hospital stay was 18.5 days. Sixty three patients required a consultation with the Rheumatology team specially due to osteoarthritis and crystal induced diseases. Conclusions: Admissions due to rheumatic diseases are prolonged, correspond to 0.46 per cent of all admissions and the main responsible disease is systemic lupus erythematosus
Sujets)
Humains , Femelle , Mâle , Adolescent , Adulte , Adulte d'âge moyen , Polyarthrite rhumatoïde/épidémiologie , Hospitalisation/statistiques et données numériques , Lupus érythémateux disséminé/épidémiologie , Polyarthrite rhumatoïde/diagnostic , Polyarthrite rhumatoïde/thérapie , /statistiques et données numériques , Lupus érythémateux disséminé/diagnostic , Lupus érythémateux disséminé/thérapie , Durée du séjour/statistiques et données numériquesRésumé
The clinical picture of primary systemic vasculitis, that cause inflammation and necrosis of vessel walls, depend on the type, size and location of involved vessels. Frequently, their clinical presentation does not reflect the specific type of vasculitis and its prognosis. Moreover, the correct diagnosis, early and adequate treatment have an important prognostic value. Recently, new diagnostic methods for systemic vasculitis have been devised. This article critically reviews and provides information for the rational use of these new imaging techniques and laboratory procedures for the diagnosis and follow up of systemic vasculitis