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10/5/2018

GLOMERULAR DISEASES
GLOMERULAR DISEASES

Peter S. Aznar, MD, FPSP, MHPE

Diagnosis of Glomerular Diseases Clinical Syndromes in Glomerular Diseases

a. Clinical Syndrome:

a1. Nephritic Syndrome

a2. Nephrotic Syndrome


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b. Renal biopsy
i.Light microscopy (LM)
ii. Immunoflurescence (IF)
PRIMARY GLOMERULOPATHIES (NEPHRITIC)
iii.Electron microscopy (EM)

1. Acute Poststreptococcal GN
b.Clinical features
i. Decreasing in incidence in the United States
ii.Children affected more frequently than adults
a. Synonyms iii.Occurs 2-4 weeks after a streptococcal
– acute proliferative GN infection of the throat or skin
– postinfectious GN iv. Organism: Hemolytic group A streptococci
v. May be caused by other bacteria, viruses, and
parasites and systemic diseases (SLE and
polyarteritis nodosa [PAN])
vi.Nephritic syndrome
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d. Light microscopy
• Laboratory studies
i. Hypercellular glomeruli with
i. Elevated antistreptolysin O (ASO) titers neutrophils and monocytes
ii. Red cell casts in the renal tubules
ii. Low serum complement
c.Immunofluorescence

i. granular deposits of IgG, IgM and C3 subepithelial area


ii. this deposits are known as “humps”

Subepithelial Humps

e. Electron microscopy

i. subepithelial (humps) immune complex deposits

f.Treatment

i. conservative fluid management


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h. Prognosis
2. Goodpasture syndrome (anti-GBM disease)

i. Children a. Pathogenesis
– Complete recovery in >95% of cases i. Production of antibodies directed against basement
– Rapidly Progressive Glomerulonephritis (RPGN) (1%)
membrane (anti-GBM ntibodies), which result in damage
– Chronic Glomerulonephritis (2%)
of the lungs and the kidney

ii.Adults
ii. The Goodpasture antigen is the noncollagenous
– Complete recovery (60%)
– RPGN/Chronic Renal Disease (40%) component of type IV collagen

b. Clinical features c.Light microscopy: hypercellularity, crescents and fibrin


i.Males > Females
d. Electron microscopy: no deposits, but there is
ii. Peak incidence: ages 20-40 years glomerular basement membrane (GBM) disruption

iii.Pulmonary involvement typically precedes e. Immunofluorescence: smooth and linear pattern of


the renal disease IgG and C3 in the GBM

iv.Present with pulmonary hemorrhage and f. Treatment: plasma exchange, steroids and cytotoxic drugs
recurrent hemoptysis

v. Most develop Rapidly Progressive Glomerulonephritis


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3. Rapidly Progressive Glomerulonephritis


g. Prognosis (RPGN)
i. Poor
a. Synonym: Crescentic Glomerulonephritis
ii. Pulmonary hemorrhage may be severe and life threatening

iii.Rapidly progressive renal failure is common


b. Clinical feature: rapid progression to severe
renal failure in weeks or months
iv. Early aggressive treatment may prevent end-stage renal failure

Crescentic GN

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