Immunotactoid glomerulopathy characterized by steroid-responsive massive subendothelial deposition.

摘要

We report a case of immunotactoid glomerulopathy with unique histologic findings in serial biopsies. A 73-year-old man complained of developing general edema. Laboratory data on admission presented moderate renal dysfunction with nephrotic syndrome. There was no evidence of systemic disease that might cause secondary glomerulopathy. Light microscopy of the renal specimen revealed lobulation of glomerular tufts and massive endothelial deposition of hyaline-like periodic acid-Schiff-positive substance with neutrophilic infiltration. The deposits were positive for immunoglobulin by immunohistochemical stains but negative for Congo red stain. Electron microscopy disclosed the deposition of microtubular structure (60 nm in diameter) predominantly in the subendothelial area and to some extent in the subepithelial and mesangial areas. Some of the tubules were extremely large (100 to 130 nm in diameter) and displayed a unique scroll structure in cross-section. The patient was treated with two sessions of plasma exchange and subsequent oral prednisolone (30 mg/d). Proteinuria and renal dysfunction improved significantly in the following 2 months. Second and third renal biopsies revealed disappearance of the deposit along with the improvement of proteinuria and renal dysfunction. Because similar microtubular structures were found in neutrophils in the glomerulus as well as in the urinary sediment, phagocytosis was suggested as a possible mechanism for removal of the deposit.