Ann Rheum Dis 1998;57:456-459 ( August )

Extended reports

Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults Josep Font,^a Ricard Cervera,^a Gerard Espinosa,^a Lucio Pallarés,^b Manel Ramos-Casals,^a Sònia Jiménez,^a Mario García-Carrasco,^a Luís Seisdedos,^a Miguel Ingelmo^a

^a Systemic Autoimmune Diseases Unit, Department of Medicine, IDIBAPS^*, Hospital Clínic, School of Medicine, University of Barcelona, Barcelona, Catalonia, Spain, ^b Department of Internal Medicine, Hospital Son Dureta, Majorca, Balearic Islands, Spain

Correspondence to: Dr J Font, Unitat de Malalties Autoimmunes Sistèmiques, Hospital Clínic, Villarroel, 170, 08036-Barcelona, Catalonia, Spain.

Accepted for publication 2 July 1998

OBJECTIVETo define the pattern of disease expression in patients with childhood onset systemic lupus erythematosus (SLE).
METHODSProspective analysis of clinical manifestations and immunological features of 34 patients in whom the first manifestations appeared in childhood from a series of 430 unselected patients with SLE.
RESULTSThirty one (91%) patients from the childhood onset group were female and three male (9%) (ratio female/male, 10/1, with no difference compared with the adult onset group). Mean age of this group at disease onset was 11 years (range 5-14) compared with 32 years (15-48) for the remaining patients. The childhood onset patients more often had nephropathy (20% v 9% in adult onset SLE, p=0.04; OR:2.7; 95%CI:1.1, 7), fever (41% v 21%, p=0.006; OR:2.6, 95%CI:1.2, 5.7), and lymphadenopathy (6% v 0.5%, p=0.03, OR: 12.3, 95%CI: 1.2, 127.6), as presenting clinical manifestations. During the evolution of the disease, the childhood onset patients had an increased prevalence of malar rash (79% v 51%, p=0.002; OR:3.7; 95%CI:1.5, 9.5) and chorea (9% v 0%, p<0.0001). This group exhibited a higher prevalence of anticardiolipin antibodies (aCL) of the IgG isotype when compared with the remaining patients (29% v 13%, p=0.017; OR:2.9, 95%CI:1.2, 6.8). No significant differences were found among the other antibodies between the two groups. Childhood onset patients more often received azathioprine (15% v 6%, p=0.00004; OR:11.2; 95%CI:2.8, 44.9) but no differences were detected between the groups concerning side effects or drug toxicity.
CONCLUSIONSThe presentation and the clinical course of SLE varied in this series of 430 patients depending on their age at disease onset. Nephropathy, fever, and lymphadenopathy were more common in childhood onset patients as presenting clinical manifestations, while malar rash, chorea, and detection of IgG aCL were more common during the evolution of the disease.

Keywords: systemic lupus erythematosus; childhood; age

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^* IDIBAPS=Institute d'Investigacions Biomèdiques August Pi i Sunyer.

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© 1998 by Annals of the Rheumatic Diseases
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