스테로이드에 반응하는 IgA 신증에 동반된 신증후군

Abstract

We are reporting four cases of steroid responsive nephrotic syndrome which showed mesangial IgA deposition at immunofluorescence. In IgA nephropathy severe proteinuria is rare and more often associated with hypertension, steroid resistance, and a tendency to renal insufficiency with severe renal histopathologic changes such as advanced glomerular sclerosis and crescent formation. But these cases exhibited minor glomerular alterations at light microscopy despite of massive proteinuria. Furthermore, these cases took a clinical course mimicking that of minimal change nephrotic syndrome, characterized by steroid responsive proteinuria, infrequent relapses (in case 2), absence of hematuria, and absence of renal insufficiency during the follow-up period. However, we could diagnose these cases by presence of mesangial IgA deposits at immunofluorescence. In conclusion, we could recommend the use of corticosteroid particularly in case of the nephrotic syndrome associated with IgA nephropathy with minor histopathologic change.

IgA 신증에서 신증후군의 동반은 예후가 좋지못한 것 으로 알려져 있으나 임상적으로 반복되는 혈뇨가 없고 신생검상 병변이 경미하면 스테로이드를 사용하여 이에 대한 반응을 보는 것이 중요하다. 미소변화형 신증후군 과 IgA 신중이 우연히 공존하는지 아니면 공통된 병인 에 의해 한 환자에서 두 질환이 발생하는지는 앞으로 보다 많은연구가있어야될것 같다.