Professional Documents
Culture Documents
glomerulopathy
Pathology
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.
Pathogenesis
MN is believed to be caused by in situ immune complex
formation.
Therefore, antigens from the infectious agents or
medications associated with secondary MN directly
contribute to the pathogenesis of the renal disease.
The causative agents or antigens in the other secondary
forms of MN have not been defined.
Likewise, the causative antigen in idiopathic MN is not
established, but the M-type phospholipase A2 receptor may
be a target antigen in idiopathic MN.
Clinical Manifestations
In children, membranous glomerulopathy is
most common in the 2nd decade of life, but it
can occur at any age, including infancy.
The disease usually manifests as nephrotic
syndrome and accounts for 2-6% of all cases of
childhood nephrotic syndrome.
Most patients also have microscopic hematuria
and only rarely present with gross hematuria.
Diagnosis
Membranous glomerulopathy might be suspected on
clinical grounds, particularly in the setting of known risk
factors for secondary forms of the disease.
The diagnosis can only be established by renal biopsy.
No serologic test is specific for MN, but finding an active
carrier state for hepatitis B or congenital syphilis would
make the diagnosis probable in the appropriate clinical
setting.
Common indications for renal biopsy leading to the
diagnosis of MN include presentation with nephrotic
syndrome in a child >10yr or unexplained persistent
hematuria with significant proteinuria.