Current recommendations for the treatment of pediatric SLE are from un
controlled trials, case reports, retrospective descriptive data or ext
ra-polation from studies in adults. Glucocorticoids are the mainstay o
f therapy and the doses depend on the disease severity. Diffuse prolif
erative glomerulonephritis (DPGN) requires high-dose prednisone for pr
olonged periods of time. We suggest the addition of azathioprine for D
PGN at the time of diagnosis of DPGN and reserve cyclophosphamide for
refractory cases. While we do not recommend the routine use of cycloph
osphamide in this or other forms of lupus nephritis, others advocate t
he aggressive use of intravenous cyclophosphamide and prednisone. Seve
re central nervous system disease should be treated with high dose pre
dnisone and immunosuppressive agents are reserved for life-threatening
disease or steroid failure or dependency. We suggest the routine use
of hydroxychloroquine in all cases of SLE at a dose of 5 mg/kg/day (ma
ximum of 400 mg/day). Methotrexate has been recently used with some su
ccess in both children and adults, the safety profile appears to be ve
ry good and therefore further studies of this drug are warranted. Coll
aboration in the development of a limited number of defined treatment
protocols and large scale collection of data on a multicenter and mult
inational basis is needed if we hope to improve the outcome of patient
s with severe disease.