BACKGROUND Immunoglobulin A nephropathy(IgAN)is the most commonly encountered glomerular disease in Asian countries.It has a broad clinical presentation,and it is frequently associated with other conditions.Chronic li...BACKGROUND Immunoglobulin A nephropathy(IgAN)is the most commonly encountered glomerular disease in Asian countries.It has a broad clinical presentation,and it is frequently associated with other conditions.Chronic liver disease is well recognized as the leading cause of secondary IgAN.However,cases of IgAN associated with autoimmune hepatitis(AIH)have seldom been reported.CASE SUMMARY A 63-year-old Korean woman was admitted to Pusan National University Hospital for an evaluation of abdominal pain and elevated liver enzymes.Two weeks prior,she had presented to our hospital with proteinuria of approximately 1350 mg/d and hematuria and was diagnosed with IgAN.Autoimmune profiles were highly positive for antinuclear antibodies,and symptoms related to portal hypertension including ascites and peripheral edema were present.A diagnosis of AIH was made according to the simplified scoring system of the International Autoimmune Hepatitis Group.Despite immunosuppression with prednisolone and azathioprine,rapid deterioration of liver function led to end-stage liver disease.After a living-donor liver transplantation,liver function gradually improved,and she had maintained stable liver and kidney function at the six months follow-up.CONCLUSION Cases of secondary IgAN with chronic liver disease have been frequently reported in the literature but are rarely associated with AIH.We encountered an IgAN patient with concurrent progressive liver failure due to AIH.展开更多
BACKGROUND Post-transplant nephrotic syndrome(PTNS)in a renal allograft carries a 48%to 77%risk of graft failure at 5 years if proteinuria persists.PTNS can be due to either recurrence of native renal disease or de no...BACKGROUND Post-transplant nephrotic syndrome(PTNS)in a renal allograft carries a 48%to 77%risk of graft failure at 5 years if proteinuria persists.PTNS can be due to either recurrence of native renal disease or de novo glomerular disease.Its prognosis depends upon the underlying pathophysiology.We describe a case of post-transplant membranous nephropathy(MN)that developed 3 mo after kidney transplant.The patient was properly evaluated for pathophysiology,which helped in the management of the case.CASE SUMMARY This 22-year-old patient had chronic pyelonephritis.He received a living donor kidney,and human leukocyte antigen-DR(HLA-DR)mismatching was zero.PTNS was discovered at the follow-up visit 3 mo after the transplant.Graft histopathology was suggestive of MN.In the past antibody-mediated rejection(ABMR)might have been misinterpreted as de novo MN due to the lack of technologies available to make an accurate diagnosis.Some researchers have observed that HLA-DR is present on podocytes causing an anti-DR antibody deposition and development of de novo MN.They also reported poor prognosis in their series.Here,we excluded the secondary causes of MN.Immunohistochemistry was suggestive of IgG1 deposits that favoured the diagnosis of de novo MN.The patient responded well to an increase in the dose of tacrolimus and angiotensin converting enzyme inhibitor.CONCLUSION Exposure of hidden antigens on the podocytes in allografts may have led to subepithelial antibody deposition causing de novo MN.展开更多
基金Pusan National University Hospital Education and Research Team,No 219。
文摘BACKGROUND Immunoglobulin A nephropathy(IgAN)is the most commonly encountered glomerular disease in Asian countries.It has a broad clinical presentation,and it is frequently associated with other conditions.Chronic liver disease is well recognized as the leading cause of secondary IgAN.However,cases of IgAN associated with autoimmune hepatitis(AIH)have seldom been reported.CASE SUMMARY A 63-year-old Korean woman was admitted to Pusan National University Hospital for an evaluation of abdominal pain and elevated liver enzymes.Two weeks prior,she had presented to our hospital with proteinuria of approximately 1350 mg/d and hematuria and was diagnosed with IgAN.Autoimmune profiles were highly positive for antinuclear antibodies,and symptoms related to portal hypertension including ascites and peripheral edema were present.A diagnosis of AIH was made according to the simplified scoring system of the International Autoimmune Hepatitis Group.Despite immunosuppression with prednisolone and azathioprine,rapid deterioration of liver function led to end-stage liver disease.After a living-donor liver transplantation,liver function gradually improved,and she had maintained stable liver and kidney function at the six months follow-up.CONCLUSION Cases of secondary IgAN with chronic liver disease have been frequently reported in the literature but are rarely associated with AIH.We encountered an IgAN patient with concurrent progressive liver failure due to AIH.
文摘BACKGROUND Post-transplant nephrotic syndrome(PTNS)in a renal allograft carries a 48%to 77%risk of graft failure at 5 years if proteinuria persists.PTNS can be due to either recurrence of native renal disease or de novo glomerular disease.Its prognosis depends upon the underlying pathophysiology.We describe a case of post-transplant membranous nephropathy(MN)that developed 3 mo after kidney transplant.The patient was properly evaluated for pathophysiology,which helped in the management of the case.CASE SUMMARY This 22-year-old patient had chronic pyelonephritis.He received a living donor kidney,and human leukocyte antigen-DR(HLA-DR)mismatching was zero.PTNS was discovered at the follow-up visit 3 mo after the transplant.Graft histopathology was suggestive of MN.In the past antibody-mediated rejection(ABMR)might have been misinterpreted as de novo MN due to the lack of technologies available to make an accurate diagnosis.Some researchers have observed that HLA-DR is present on podocytes causing an anti-DR antibody deposition and development of de novo MN.They also reported poor prognosis in their series.Here,we excluded the secondary causes of MN.Immunohistochemistry was suggestive of IgG1 deposits that favoured the diagnosis of de novo MN.The patient responded well to an increase in the dose of tacrolimus and angiotensin converting enzyme inhibitor.CONCLUSION Exposure of hidden antigens on the podocytes in allografts may have led to subepithelial antibody deposition causing de novo MN.
