Clinical and serological features of juvenile systemic lupus erythematosus in an italian tertiary centre of pediatric rheumatology

Results Fever and fatigue were frequent symptoms at onset, occurring respetively in 47 (45.1%) and 39 (37.5%) patients. The most common organ involvement was skeletal, affecting 89 (85.5%) of patients, 87 (97.7%) of whom were affected by non erosive arthritis. Jaccoud’s arthropathy was observed in 7 (6.7%) patients. Malar rash, leukopenia and non scarring alopecia were common findings. They occurred respectively in 60 (57.6%), 59 (56.7%) and 52 (50%) patients. Clinical evidence of renal involvement occurred in 42 (40.3%) patients. 31 patients had 1 or more renal biopsies: 22 (70.9%) resulted affected by diffuse proliferative glomerulonephritis, 6 (19,3%) by focal proliferative glomerulonephritis and 6 (19.3%) by membranous nephropathy. Cutaneous vasculitic lesions, observed in 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.