Abstract
Recent studies suggest that galactose-deficient IgA1 (Gd-IgA1) plays a role in the pathogenesis of primary IgA nephropathy (IgAN) and Henoch–Schönlein purpura nephritis (HSPN). Furthermore, immunostaining of KM55, an antibody that identifies Gd-IgA1, may be helpful to differentiate primary IgAN and HSPN from secondary causes of glomerular IgA deposition. We report sequential kidney biopsies of a malignancy-associated HSPN, showing intense glomerular mesangial IgA deposition at the initial kidney biopsy and dramatic decrease in disappearance of glomerular deposits after tumor removal. We demonstrate that the glomerular IgA deposition contains Gd-IgA1, detected by immunostaining of KM55, with similar distribution and intensity to IgA. This suggests that renal Gd-IgA1 deposition may play a role in the pathogenesis of malignancy-associated HSPN.
Highlights
Henoch–Schonlein purpura (HSP), referred as immunoglobulin A vasculitis (IgAV), is the most common systemic vasculitis involving the small blood vessels in pediatric population with kidney involvement present in 30–50% [1]
Recent study shows KM55 is detected in primary IgA nephropathy (IgAN) and Henoch–Schonlein purpura nephritis (HSPN), but not in other examined renal diseases, such as lupus nephritis, cirrhosis, or hepatitis C-related glomerular disease [6]. ese findings suggest IgAN and HSPN share a common feature involving GdIgA1 in pathogenesis [6]
E renal manifestations of HSPN can range from microscopic hematuria and proteinuria only to a rapidly progressive glomerulonephritis (RPGN)
Summary
Henoch–Schonlein purpura (HSP), referred as immunoglobulin A vasculitis (IgAV), is the most common systemic vasculitis involving the small blood vessels in pediatric population with kidney involvement present in 30–50% [1]. Similar to IgA nephropathy (IgAN), Henoch–Schonlein purpura nephritis (HSPN) is characterized by the deposition of IgA dominant or codominant immune complex in the glomerular mesangial and capillary wall regions. Recent study shows KM55 is detected in primary IgAN and HSPN, but not in other examined renal diseases, such as lupus nephritis, cirrhosis, or hepatitis C-related glomerular disease [6]. Case Reports in Nephrology neoantigens leading to the formation of immune complexes and similarities between tumor antigens and endothelial cell antigens. It is unknown whether Gd-IgA1 plays a role in the pathogenesis of malignancy-associated HSPN. We report detailed clinical pathological findings and immunostaining of KM55 on sequential kidney biopsies of a 35-year-old man with a clear cell renal cell carcinoma(CCRCC-) associated HSPN
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