Published on 22/03/2015 by admin
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Chapter 506 Membranous Glomerulopathy
Scott K. Van Why, Ellis D. Avner
Membranous glomerulopathy, now commonly called membranous nephropathy (MN), a common cause of nephrotic syndrome in adults, is a rare cause of nephrotic syndrome in children. MN is classified as the primary, idiopathic form, where there is isolated renal disease, or secondary MN, where nephropathy is associated with other identifiable systemic diseases or medications. In children, secondary MN is far more common than primary, idiopathic MN. The most common etiologies of secondary MN are systemic lupus erythematosus (SLE) or chronic infections. Among the latter, chronic hepatitis B infection and congenital syphilis are the best characterized and widely recognized causes of MN. However, other chronic infections have also been associated with MN, including malaria, which is the most common cause of MN worldwide. Certain medications, such as penicillamine and gold, can also cause MN. Rarely, tumors, such as neuroblastoma, or other idiopathic systemic diseases have been associated with MN. Identification of secondary causes of MN is critical, because removal of the offending agent or treatment of the causative disease often leads to resolution of the associated nephropathy and improves patient outcome.
Glomeruli have diffuse thickening of the glomerular basement membrane (GBM), without significant cell proliferative changes. Immunofluorescence and electron microscopy typically demonstrate granular deposits of IgG and C3 located on the epithelial side of the GBM in a spikelike pattern. The GBM thickening presumably results from the production of membrane-like material in response to deposition of immune complexes.
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