Primary membranous nephropathy in adolescence: A prospective study.

AIM

Primary membranous nephropathy (PMN) accounts for only 1-2% of nephrotic syndrome in children. Antibodies to m-type phospholipase A2 receptor (aPLA R) is seen in 70% of adult PMN cases. The present study was undertaken to study m-type phospholipase A receptor (PLA R) status and clinical behavior in adolescent PMN cases.

METHODS

The present prospective observational study included adolescent (10-19 years) onset biopsy proved PMN. Patients were followed on a monthly basis with urine protein, serum albumin and creatinine. Serum aPLA R was done at baseline and at 6 and 12 months of starting treatment. Patients were treated as per unit's protocol.

RESULTS

During the study period a total of 18 patients were enrolled. The mean age of the cases was 16.27 ± 2.39 (11-19) years. Seventeen (94.44%) patients presented with nephrotic syndrome. The mean proteinuria and serum albumin was 4.52 ± 1.93 (2.43-9.20) g/day and 2.1 ± 0.6 (1.1-3.4) g/dL respectively. PMN was PLA R related in 83%. aPLA2R and enhanced staining for PLA R in glomeruli was seen in 14 (77.78%) and 13 (72.22%), respectively. Clinical remission at the end of 6 and 12 months of therapy was seen in 11 (61.11%) and 9 (50%) subjects respectively. There was a significant association of aPLA R to clinical remission/ resistance.

CONCLUSION

Primary membranous nephropathy in adolescent population is aPLA R related in over three-quarters of the cases and the response to therapy is seen in only half of them. aPLA R monitoring is clinically relevant and should be incorporated in the management of adolescent onset PMN.