IMMUNOSUPPRESSION
MANAGEMENT IN CHILDHOOD GLOMERULOPATHIES
Burke J R
Mater and Royal Childrens’ Hospitals, Brisbane,
Australia
Decisions on appropriate immunosuppressive therapy
in lupus erythematosis, Henoch Schölein purpura, membranoproliferative
glomerulonephritis, crescentic post streptococcal glomerulonephritis are
often difficult due to the lack of randomised controlled trials.
Lupus – In focal proliferative
nephritis (stage II & III) there is mild proteinuria and treatment is
limited to extra renal symptomatology.
In diffuse proliferative nephritis (stage IV)
approximately 20% progress to renal failure. Prednisolone with cyclophosphamide or azathioprine gives
a better prognosis than prednisolone alone. In crescentic form treatment includes methylprednisolone
1g/1.73m2/day for 3 days, oral prednisolone 1.5-2mg/kg and
cyclophosphamide IV 500-1,000 mg/m2/month or oral 2mg/kg/day for
6 months. The risk of
infertility with cyclophosphamide is variable depending on age, dose and
gender, and should be reviewed after 6 months treatment. Mycophenolate with prednisolone has
been shown to be as effective as prednisolone and cyclophosphamide followed
by prednisolone and azathioprine over a 12-month period. Infertility and amenorrhoea may be
reduced.
In membranous nephropathy (stage V) with nephrotic
syndrome cyclosporin with prednisolone may have less side effects than
prednisolone and an alkalating agent.
Henoch Schölein purpura – Prevention of clinical
IgA nephropathy has been attempted in 4 studies with prednisolone
1-2.5mg/kg/day for 7 – 21 days.
Some showed a decrease in development of haematuria but no evidence
for prevention of chronic renal failure.
Approximately 3% develop chronic renal
failure. In mild disease there
is no evidence that immunosuppression is indicated. In severe disease (>50%
crescents, nephrotic syndrome) treatment often includes methylprednisolone
1g/1.73m2 daily for 3 days followed by oral prednisolone,
cyclophosphamide for 3 months followed by azathioprine. However there are no controlled
trials.
Membranoproliferative glomerulonephritis (Type
I,II,III) – A number of uncontrolled studies show some patients treated
with high dose alternate day prednisolone (2mg/kg) have a better outcome
than historical controls.
There is no proven advantage for the addition of cyclophosphamide,
cyclosporin or plasmapheresis.
Post streptococcal glomerulonephritis –
crescentic glomerulonephritis is common in post infectious nephritis and
approximately 95% make a complete recovery. In rapidly progressive glomerulonephritis there are case
reports of recovery of renal function with methylprednisolone, azathioprine
and plasmapheresis.