Systemic lupus erythematosus in childhood - an update

Approximately 20 percent of all cases of systemic lupus erythematosus (SLE) begin prior to the age of 18. The most characteristic feature is malar rash. oral or nasal ulcers, photosensitivity and discoid lesions are also typical. The arthritis of SLE tends to be symmetrically polyarticular with a favourable prognosis. Pleuritis or pericarditis are quite common in paediatric patients. More severe cardiac or pulmonary manifestations are less frequent but may be life-threatening. Neuropsychiatric manifestations are present in 10-20 % of patients and may require aggressive therapy. Nephritis is one of the most serious complications of SLE and occurs in 60-65 % of paediatric patients. All patients with signs of renal disease should have a renal biopsy which then directs therapy. The most severe form of lupus nephritis, class IV nephritis occurs in about 40-50% of paediatric patients with nephritis. Endocrine involvement is very common, particularly abnormalities of thyroid function. Growth disturbances may be secondary to the chronic illness and/or the use of corticosteroids, but growth hormone deficiency also occurs in patients with SLE. Haematological abnormalities concern haemolytic anaemia, thrombocytopenia and leucopenia. Specific autoantibodies are diagnostic for SLE. The hallmark are anti-DNA antibodies which are present in 80-85 % of paediatric patients. Anti-phospholipid antibodies are associated with thrombosis which in paediatric patients is venous rather than arterial. Patients with a severe headache should be investigated for cerebral vein thrombosis. Patients with pediatric SLE tend to have a more severe disease than that seen in adults. Corticosteroids remain the mainstay of therapy, but they are associated with a significant morbidity and may lead to premature atherosclerosis. In patients with significant organ manifestation early introduction of immunosuppressive agents, for children preferably azathioprine, should be considered.