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Glomerular Diseases Glomerular Diseases Glomerular Diseases: Peter S. Aznar, MD, FPSP, MHPE
Glomerular Diseases Glomerular Diseases Glomerular Diseases: Peter S. Aznar, MD, FPSP, MHPE
Glomerular Diseases Glomerular Diseases Glomerular Diseases: Peter S. Aznar, MD, FPSP, MHPE
GLOMERULAR DISEASES
GLOMERULAR DISEASES
a. Clinical Syndrome:
b. Renal biopsy
i. Light microscopy (LM)
ii. Immunoflurescence (IF)
PRIMARY GLOMERULOPATHIES (NEPHRITIC)
iii. Electron microscopy (EM)
d. Light microscopy
• Laboratory studies
i. Hypercellular glomeruli with neutrophils and
i. Elevated antistreptolysin O (ASO) titers monocytes
ii. Red cell casts in the renal tubules
ii. Low serum complement
c. Immunofluorescence
Subepithelial Humps
e. Electron microscopy
f. Treatment
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
– Complete recovery (60%)
ii. The Goodpasture antigen is the noncollagenous
– RPGN/Chronic Renal Disease (40%)
component of type IV collagen
iii. Pulmonary involvement typically precedes the renal e. Immunofluorescence: smooth and linear pattern of IgG and C3 in
disease the GBM
iv. Present with pulmonary hemorrhage and recurrent f. Treatment: plasma exchange, steroids and cytotoxic drugs
hemoptysis
Crescentic GN
10/5/2018
v. Idiopathic
Types of RPGNs
f. Electron microscopy
• Type I (25%)
i. Variable
– anti-GBM +
ii. May or may not have electron-dense deposits
– May have a pulmonary component
iii. GBM disruption and discontinuity is commonly seen
(Goodpasture syndrome)
– High prevalence of HLA subtypes
f. Prognosis
i. poor with rapid progression to acute renal failure and
end-stage renal disease
10/5/2018
a. Clinical features
a. Epidemiology
iv. Recurrent gross hematuria
i. Most common cause of glomerulonephritis in the world
v. Onset may follow a respiratory infection
ii. Common in France, Japan, Italy and Austria
vi. Predominantly nephritic
iii. Affects children and young adults (mostly males)
vii. Associated with Celiac Sprue and Henoch-Schonlein
purpura
10/5/2018
b. Pathogenesis
d. Immunofluorescence: mesangial deposits of IgA and C3
i. The mechanism is unknown. There is a possible
entrapment of circulating immune complexes with e. Electron microscopy: mesangial immune complex
activation of the alternate complement pathway. There is deposits
also a possible genetic predisposition.
f. Prognosis: many cases slowly progress to renal failure
c. Light microscopy over 25 years
i. Variable
ii. Normal or mesangial proliferation
d. Light microscopy
i. Lobulated appearance of the glomeruli
ii. Mesangial proliferation and basement-membrane
thickening
iii. Splitting of the basement membrane (“tram-tracking”) may be
seen with a silver or periodic acid-Schiff (PAS) stain
e. Immunofluorescence
i. Type I: granular pattern of C3 often with IgG, C1q and g. Prognosis
C4
ii. Type II: granular and linear pattern of C3
i. Slowly progressive course, resulting in chronic renal
failure over the course of 10 years
f. Electron microscopy
i. Type I: subendothelial and mesangial immune ii. High incidence of recurrence in transplants
complex deposits
ii. Type II: dense deposits within the GBM
10/5/2018
6. Alport Syndrome