Korean Journal of Nephrology 1994;13(3):547-559.

IgA 신병증과 Henoch - Schonlein 자반증 신염의 임상 및 병리학적 비교 연구

이수경 , 권민중 , 이재승 , 김병길 , 임현이 , 최인준

Abstract

IgA nephropathy (IgAN) and Henoch-Schonlein purpura nephritis (H-SPN) are both characterized by mesangial deposition of immu@ne reactants. H-SPN is accompanied by systemic symptoms such as purpuric skin rashes, abdominal pain and arthralgia. Occa- sionally IgAN is not distinguished with H-SPN in clini- cal and pathologic aspects. This facts suggest that H- SPN and IgAN can be genetically related, therefore supporting the notion that these two disease entities probably share a common pathogenesis. Many reports revealed that IgAN and H-SPN can progress to nephrotic syndrome and chroinc renal failure. A few cases involved renal transplatation reported also. 62 patients were proved as having IgAN and were observed by clinical, laboratory and pathologic method and compared with 56 cases of H-SPN. There was no statistical significance in sex ratio, duration of disease, incidence of hypertension, renal function and serum level of IgA. There was significance difference in the incidence of gross hematuria at the time of disease onset: 47 cases (76.8%.) in IgAN and 13 cases (13.3%.) in H-SPN. Urine protein above 1 g/day was found in 18 cases (29.0%,) in IgAN and 29 cases (51.8%) in H-SPN, but no statistical significance. Urine protein above 3 g/day was demon- strated in 8 cases in IgAN and 15 cases in H-SPN. In the two groups mentioned above, gross hematuria was do umented in all 8 cases in IgAN but only 5 cases (33.3%,) in H-SPN making a statistical significance. There was a tendency of an increase of urine protein in proportion to the pathologic grading in both disease entities. Immunofluorscent study revealed a statistical significance by showing a higher rate of fibrinogen deposion in the mesangium and IgA, IgG and fibrinogen depostion in the capillary in H-SPN. Cyclosporin A may have a therapeutic role in the treatment of IgAN and H-SPN, particularly with nephrotic syndrome. Among the patients with H-SPN, one case received kidney transplantation 8 years after diagnosis. Because the patients with H-SPN revealed more severe proteinuria and immune deposition in the kidney, even having one patient experiencing kidney transplantation, continuous follow up and active treatment is required in case of nephrotic syndrome. However, the difficulty is the H-SPN is not distinguished with IgAN if H-SPN is not confirmed by history and physical examination.