Abstract
38 (36.5%) patients, were an important cause of morbidity. Oral ulcers and serositis were found in 27 (25.9%) and 25 (24%) patients respectively. Neurologic involmentent occurred in 20 (19.2%) patients. Seizures were observed in 8 (7.6%) patients. Infections remain a major problem in morbidity: serious infective manifestations occurred in 21 (20.1%) patients. Avascular necrosis of bone occurred in 7 (6.7%) patients, 3 of whom suffered more than 1 episode. Six (5.7%) patients developed steroid induced cataract. Tendon ruptures were observed in 4 (3.8%) patients. Growth failure and established osteoporosis, resulting from prolonged corticosteroid treatment in chronically active disease, were unfrequent but severe complications in our jSLE series occurring respectively in 8 (7.6%) and 5 (4.8%) patients. Cushingoid features and striae rubrae were frequent problems observed in 17 (16.3%) patients, due to disease itself and/or its treatment, that caused increased psychological distress, particularly in adolescents. Finally there were 4 deaths. Two due to infection complications and two due to myocardial infarction.
Highlights
Juvenile systemic lupus erythematosus is a multisystem autoimmune disorder that is characterized by widely variable clinical presentations and uncertain course
104 patients affected by Juvenile systemic lupus erythematosus (jSLE) (< 18 years) were enrolled in thirty one years. 11 patients were male
Fever and fatigue were frequent symptoms at onset, occurring respetively in 47 (45.1%) and 39 (37.5%) patients
Summary
Juvenile systemic lupus erythematosus (jSLE) is a multisystem autoimmune disorder that is characterized by widely variable clinical presentations and uncertain course. Clinical and serological features of juvenile systemic lupus erythematosus in an Italian tertiary centre of pediatric rheumatology Introduction Juvenile systemic lupus erythematosus (jSLE) is a multisystem autoimmune disorder that is characterized by widely variable clinical presentations and uncertain course. Objectives To evaluate the clinical and serological features of jSLE in a long lasting observation of a single italian centre.
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