ESPN 51th Annual Meeting

ESPN 2018


 
Long follow-up of Lupus Nephropathy with onset in childhood.
MAR ESPINO-HERNANDEZ 1 JULIA VARA-MARTIN 1 TANIA CARBAYO JIMENEZ 1 NELLY ROMAN MENDOZA 1 MARINA ALONSO RIAÑO 2 JUSTINO JIMENEZ ALMONACID 2 ALBERTO SERVAN 1 CRISTINA LILLO 1 SERGIO FOULLERAT 1

1- Pediatric Nephrology. Hospital 12 de Octubre. Madrid. Spain.
2- Pathology. Hospital 12 de Octubre. Madrid. Spain.
 
Introduction:

To know the outcome and prognosis in adulthood of lupus nephropathy (LN) with onset in infancy.

Material and methods:

Retrospective study of patients with diagnosis of LN in renal biopsy. We recorded age, clinical manifestations, hypertension (HTA), glomerular filtration rate, proteinuria, hematuria, histopathology and treatment, at onset and at the time of transition to adulthood.

Results:

We included nineteen patients (16 girls 3 boys), mean age at onset 11 years (range 6-14 y). Initial presentation was: hypertension 6, renal failure 7, proteinuria 18 (nephrotic syndrome 8), hematuria 14. Histopathology showed: Class II LN (mesangial proliferation) 4, Class III (focal proliferation) 3, Class IV (diffuse proliferation) 9 and Class V (membranous) 2 and one inconclusive. Initial treatment was: prednisone (P) and cyclophosphamide (CYC) IV 13 patients, P and mycophenolate (MF) 3, only prednisone 2 (but later required CYC treatment), and azathioprine with prednisone in one. We associated chloroquine in 6. At the time of transition to adults, mean age 17 (range 14-18), mean follow-up 6 years (range 4-8): thirteen patients were in remission (4 without treatment, 4 on P and five on MF/P treatment), three were partial remission on CYC/P, two were in relapse on CF/P and renal failure, one progressed into chronic renal failure with renal transplantation. Second renal biopsy was performed in three patients, two to stop CYC treatment (one changed class IV to II and the other was inconclusive), the third was done because not remission (changed from class II to IV).

Conclusions:

In childhood, the most typical presentation of lupus nephropathy is proteinuria. Clinical manifestations correlate poorly with renal biopsy, so in some cases it may be necessary to re-biopsy. In this historical series remission on cyclophosphamide were achieved with a high chance of preserving normal glomerular filtration rate. In recent years the treatment with mycophenolate is safe and maintain long-term remissions.