Background Juvenile Systemic Lupus Erythematosus (JSLE) is a worldwide disease. Several studies have been made characterising the prevalence of clinical and laboratory features. However, differences in the disease pattern among different populations have been previously suggested.
Methods We have conducted a retrospective study of JSLE patients followed-up at the Rheumatology Department of Santa Maria Hospital. Clinical records of twenty-five Portuguese patients satisfying the 1982 revised ARA criteria for Systemic Lupus Erythematosus (SLE) were reviewed.
Results Twenty-three (92%) were females and two (8%) were males. The mean age of disease onset was 11.8 ± 3.6 (2.5–16) years and the mean age of diagnosis was 12.9 ± 3.6 (6–19) years. The mean follow up time was 6.5 ± 3.5 (0.5 to 14) years. The most common cumulative clinical features were arthritis in 21 patients (84%), malar rash in 19 (76%), renal involvement in 14 (56%), oral ulcers in 13 (52%), vasculitis in 13 (52%), photosensitivity in 11 (44%), central nervous system involvement in 9 (36%), livedo reticularis in 9 (36%), hypertension in 8 (32%), serositis in 5 (20%), renal failure in 5 (20%) and secondary Sjögren syndrome in 1 (4%). Eighteen patients (76%) had abnormal blood counts and seven (28%) had secondary antiphospholipid antibody syndrome. In the immunology evaluation 25 (100%) patients tested positive for antinuclear antibodies, 20 (80%) patients had hypocomplementemia, 21 (84%) patients had raised antinative dsDNA antibody, 9 (36%) had positive anti-cardiolipin antibodies, 6 (24%) had positive lupus anticoagulant, 5 (20%) patients had positive anti-Sm antibodies, 4 (16%) patients had positive anti-SSA/Ro antibodies, 4 (16%) had positive anti-RNP antibodies and 3 (12%) had positive anti-SSB/La antibodies. All the seven antiphospholipid antibody syndrome patients had positive anticardiolipin antibodies and five had positive lupus anticoagulant. The mean age of SLE onset in patients with central nervous system involvement was 10.3 ± 4.5 (2.5–16) years. The main symptoms were seizures (4 patients), headache (3 patients), organic brain syndrome (2 patients), corea (1 patient) and optic neuritis (1 patient). The mean age of SLE onset in patients with renal involvement was 12.3 ± 3.1 (6–16) years. Five (35.7%) developed renal failure. Currently, 64.7% patients are being treated with corticosteroids, 35.3% are taking antimalarials, 29.4% azathioprine and 5.9% mycophenolate mofetil. 35.3% are not taking any medication.
Conclusion The overall clinical and serological pattern of JSLE involvement was similar to other European studies. However, we found a higher female/male ratio (11.5:1) and a lower incidence of central nervous system and renal involvement than in previous reports. In comparison with the results of a study performed in our adult SLE patients (1), the juvenile patients had more frequently haematological (72/50.2%), renal (56/26.7%) and central nervous system (36/10.6%) involvement. Vasculitis (52/18.8%), antiphospholipid antibody syndrome (28/4.3%) and cutaneous (76/60%) involvement were also more frequent. We observed a lower incidence of articular involvement (84/91%) and secondary Sjögren syndrome (4/11.4%) in our juvenile population.
Rheumatology Europe 1998; 27(Suppl 2):142
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