(Type ICrGN) Anti-Glomerular

ular Basement Membrane Antibody12% of cases

- It's characterized by linear deposits of IgG and, in many cases, C3 on the GBM
- Goodpasture syndrome
anti-GBM
GBM antibodies bind to pulmonary alveolar capillary basement membranes to produce the clinical picture of : - pulmonary hemorrhages.
- associated with renal failure.
In idiopathic cases, renal involvement occurs in the absence of pulmonary disease.
-In
- Anti-GBM antibodies are present in the serum and are helpful in diagnosis.
- These
hese individuals benefit from plasmapheresis, which removes pathogenic antibodies from the circulation
circulation.
(Type II CrGN )immune complex-mediated mediated disorders44% of cases
- This can be a complication of any of the immune complex nephritides, including
including:
1- poststreptococcal GN,
2- SLE,
3- IgA nephropathy

- immunofluorescence studies reveal the characteristic granular ("lumpy bumpy") pattern of staining of the GBM and/or mesangium .
- These individuals cannot usually be helped by plasmapheresis.
(Type I CrGN) Pauci-Immune 44 % of cases
- It's defined by the lack of anti-GBM
GBM antibodies immune complex deposition detectable by immunofluorescence and electron microscopy.

- Most of these individuals have antineutrophil cytoplasmic antibodies in the serum, which have a role in some vasculitides.

- In some cases ,pauci-immune

immune CrGN is a component of a systemic vasculitis
vasculitissuch as microscopic polyangiitis or Wegener granulomatosis.

- In Other cases , immune CrGN is limited to the kidney and is thus called idiopathic.

- Clinical Course :
The onset :is
is much like that of the nephritic syndrome except that the oliguria and azotemia are more pronounced.
Proteinuria may approach rangess of nephrotic syndrome
syndrome.
Some of these persons become :anuric
anuric and require long
long-term dialysis or transplantation.

- The prognosis:(depends on the number of crescents )

Those
hose with crescents in less than 80% of the glomeruli have a be5er prognosis than those with higher percentages of crescents.
crescents

N.B. Plasma exchange benefits some individuals, particularly those with anti
anti-GBM
GBM antibody GN and Goodpasture syndrome.