LUPUS NEPHRITIS IN CHILDHOOD AND ADOLESCENCE

Lupus nephritis in childhood usually presents after the age of 10 year s, and presentation under 5 years is very rare. More males (F:M ratio 4.5:1) are affected than in adult-onset cases, but the ratio is the sa me in prepubertal and pubertal children. The incidence of clinically e vident renal disease is greater at onset than in adults (82%), the usu al presentation being with proteinuria, 50% having a nephrotic syndrom e. Half the children show World Health Organisation class IV nephritis in renal biopsies. Neuropsychiatric lupus is present at onset in 30%, may complicate 50% at some point and remains a major problem. Prognos is has improved greatly over the past 30 years, at least in part the r esult of immunosuppressive treatment. Treatment of the initial phase m ay be guided by the severity of the renal biopsy appearances, more agg ressive treatment including cytotoxic agents, i. v. methylprednisolone and perhaps plasma exchange, although the value of exchange is not es tablished. Controversy persists as to the most effective cytotoxic tre atment in the acute phase, both oral and i. v. cyclophosphamide and az athioprine being used in different units. In the chronic maintenance p hase it seems established both clinically and histologically that addi tion of a cytotoxic agent improves outcome, but again the drug and rou te of administration are contentious. Azathioprine has the advantage o f being safe for pregnancy and not gonadotoxic, whilst i. v. cyclophos phamide has been demonstrated to improve results over prednisolone alo ne in controlled trials and has advantages in non-compliant patients. No trial comparing the two regimes has been carried out, and one is ne eded. Today children much less commonly go into renal failure, and the main causes of actual death (15% of patients over 10 years) are now i nfections and extra-renal manifestations of lupus, principally neurolo gical. Morbidity of the disease and the treatment remain a major probl em, especially when treatment exacerbates complications of the disease itself, such as infections, osteonecrosis, thrombosis, vascular disea se and possibly neoplasia.