Childhood-onset lupus nephritis: a single-center experience of pulse intravenous cyclophosphamide therapy.
BACKGROUND
Evidence is accumulating about the efficacy of pulse intravenous (iv) cyclophosphamide (pCy) treatment for lupus nephritis (LN), but concern still exists on the use of this drug in children, on account of its oncogenic potential and gonadal toxicity. Medical records of 33 LN children were retrospectively analysed in order to assess the effect of treatment with pCy and corticosteroids (Cs) on renal survival and child growth.


METHODS
From 1974 to 1999, 33 pediatric patients with LN were admitted to our hospital. Clinical and hematological data were recorded for a mean period of eight years (range 1.5-18.9). Two groups of children who received different treatment protocols were compared: 19 were treated with Cs alone or combined with azathioprine (Aza) and 14 received Cs and pCy (0.5 g/m2 monthly); the mean number of Cy infusions was 13 (range 6-27).


RESULTS
In the pCy treated group, survival was better, protection of renal function lasted longer, and there were no evident short- and long-term side effects. pCy treated children showed better growth than the other group. Many important factors could have contributed to these positive effects, such as the time of onset of the disease, its duration before referral to the pediatric nephrology unit, year at first admission (mean 1985 Cs +/- AZA group vs 1988 pCy group), renal failure at onset, degree of renal lesion (renal histology not evaluated in 36% of cases).


CONCLUSIONS
pCy treatment in pediatric LN may improve patient and renal survival and seems safe, causing less growth impairment.
Barbano
Giancarlo
G
Nephrology Department, G. Gaslini Children's Hospital, Genoa, Italy. giancarlobarbano@ospedale-gaslini.ge.it
Gusmano
Rosanna
R
Damasio
Beatrice
B
Alpigiani
Maria Giannina
MG
Buoncompagni
Antonella
A
Gattorno
Marco
M
Perfumo
Francesco
F
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D017668:Age of Onset; D001379:Azathioprine / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000009:adverse effects / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D007262:Infusions, Intravenous; D008181:Lupus Nephritis / Q000175:diagnosis / Q000188:drug therapy*; D008297:Male; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D001379:Azathioprine