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Pathology
MPGN is defined by the histologic pattern of glomeruli as seen by light,
immunofluorescence, and electron microscopy.
Two subtypes have been defined on histologic criteria and are associated
with different clinical phenotypes.
Type I MPGN is most common. Glomeruli have an accentuated lobular
pattern from diffuse mesangial expansion, endocapillary proliferation, and
an increase in mesangial cells and matrix.
The glomerular capillary walls are thickened, often with splitting from
interposition of the mesangium.
Crescents, if present, indicate a poor prognosis. Immunofluorescence
microscopy reveals C3 and lesser amounts of immunoglobulin in the
mesangium and along the peripheral capillary walls in a lobular pattern.
Electron microscopy confirms numerous deposits in the mesangial and
subendothelial regions.
Pathogenesis
Because the histology of type I MPGN produced by
primary and secondary forms is indistinguishable, it
appears that this form occurs because circulating
immune complexes become trapped in the
glomerular subendothelial space, which then causes
injury, resulting in the characteristic proliferative
response and mesangial expansion.
Further evidence confirming this pathway to
glomerular injury is the finding of complement
activation through the classical pathway in as many
as 50% of affected patients.
Clinical Manifestations
MPGN is most common in the 2nd decade of life.
Systemic features could provide clues to which type of
MPGN may be present in the secondary forms of the
disease, but the 2 histologic types of idiopathic MPGN are
indistinguishable on clinical grounds.
Patients present in equal proportions with nephrotic
syndrome, acute nephritic syndrome (hematuria,
hypertension, and some level of renal insufficiency), or
persistent asymptomatic microscopic hematuria and
proteinuria.
Serum C3 complement levels are low in the majority of
cases
Differential Diagnosis
The differential diagnosis includes all forms of acute and chronic
glomerulonephritis, including idiopathic and secondary forms, along
with postinfectious glomerulonephritis.
Postinfectious glomerulonephritis, far more common than MPGN,
usually does not have nephrotic features but typically has hematuria,
hypertension, renal insufficiency, and transient low C3 complement
levels, all features that may be seen with MPGN.
In contrast to MPGN, where C3 levels usually remain persistently low,
C3 returns to normal within 2 months after the onset of postinfectious
glomerulonephritis.
The diagnosis of MPGN is made by renal biopsy.
Indications for biopsy include nephrotic syndrome in an older child,
significant proteinuria with microscopic hematuria, and
hypocomplementemia lasting >2mo in a child with acute nephritis.