glomerular diseases dr. atapour differential diagnosis and evaluation of glomerular disease
TRANSCRIPT
Glomerular Diseases
Dr. Atapour
Differential diagnosis and evaluation of
glomerular disease
CLINICAL PATTERNS OF GLOMERULAR DISEASE
• Nephrotic
• Nephritic
Nephrotic pattern
• The nephrotic proteinuria that is usually above 3.5
g/day
• Lipiduria
• Edema
• Hyperlipidemia
• Some patients also have microscopic hematuria
• Red cell casts
• Asymptomatic proteinuria
• Full-blown nephrotic syndrome
The bland sediment
• Absence of immune complex deposition in
most of these disorders:
– Minimal change disease
– Focal segmental glomerulosclerosis
– Diabetic nephropathy
– Amyloidosis.
• Lack of inflammation
• The serum creatinine concentration
Acute kidney injury in the nephrotic syndrome
• Concurrent acute tubular necrosis
• Usually in minimal change disease
Nephritic pattern
• Presence of red cells
• White blood cells
• Red cell – Dysmorphic appearance – Acanthocytes
• Mixed cellular casts
• Variable degrees of proteinuria
dysmorphic RBCs muddy brown casts
tubular cell casts oval fat body
RBC cast Hyaline cast
Pathologic classification
• Light microscopy
• Focal
• Diffuse
– This terminology is most often used in lupus
nephritis
– ANCA-positive vasculitis.
Limitations of this classification
• Same pathology and multiple causes
• Nonspecific nature of the histologic patterns
MPGN
Can be a systemic immune complex disease
– To infective endocarditis
– Systemic lupus erythematosus
– Hepatitis C virus complement
Membranous nephropathy
• Systemic lupus erythematosus
• Chronic hepatitis B virus infection
• Underlying malignancy
• Drugs
Focal segmental glomerulosclerosis
• Primary condition
• Secondary change induced by intraglomerular
hypertension or healing of previous
glomerular injury.
Nephrotic syndrome without a nephritic sediment
• Mild hematuria
• No red cell casts
Depend on age
What do you think?
Less than 15 years:
–Minimal change disease
– Focal segmental glomerulosclerosis
–Mesangial proliferative glomerulonephritis
15 to 40 years :
–Minimal change disease
–Membranous nephropathy
– Focal segmental glomerulosclerosis
–Diabetic nephropathy
– Preeclampsia
–Postinfectious glomerulonephritis
Greater than 40 years:
– Focal segmental glomerulosclerosis
– Membranous nephropathy
– Diabetic nephropathy
– Minimal change disease
– IgA nephropathy
– Primary amyloidosis
– Benign nephrosclerosis
– Postinfectious glomerulonephritis
Mild glomerulonephritis
• Mild glomerulonephritis is defined as a
nephritic sediment and:
– Normal or near normal estimated GFR
– Do not have the nephrotic syndrome
Less than 15 years
– Mild postinfectious glomerulonephritis
– IgA nephropathy
– Thin basement membrane disease
– Hereditary nephritis
– Henoch-Schönlein purpura
– Mesangial proliferative glomerulonephritis
15 to 40 years
• IgA nephropathy
• Thin basement membrane disease
• Lupus nephritis, hereditary nephritis
• Mesangial proliferative glomerulonephritis
Greater than 40 years
– IgA nephropathy
Moderate to severe glomerulonephritis
• Moderate to severe glomerulonephritis is
defined as a nephritic sediment
– Reduced estimated GFR
– Nephrotic syndrome ±
Less than 15 years
–Postinfectious glomerulonephritis
–Membranoproliferative glomerulonephritis
15 to 40 years
– Postinfectious glomerulonephritis
– lupus nephritis
– RPGN (crescentic glomerulonephritis)
– IgA nephropathy
– Fibrillary glomerulonephritis
– Membranoproliferative glomerulonephritis
Greater than 40 years :
–Rapidly progressive glomerulonephritis
–Vasculitis
– IgA nephropathy
– Fibrillary glomerulonephritis
–Postinfectious glomerulonephritis
Effect of race
In black patients :
– Focal segmental glomerulosclerosis (57 percent)
– Membranous nephropathy (24 percent)
– Minimal change disease (14 percent)
– Membranoproliferative glomerulonephritis, IgA
nephropathy, and immunotactoid nephropathy
were each present in 1 to 2 percent.
In white patients:
– Membranous nephropathy (36 percent),
– Focal segmental glomerulosclerosis (23 percent)
– Minimal change disease (20 percent)
– IgA nephropathy (8 percent)
– Membranoproliferative glomerulonephritis (6%)
– Immunotactoid glomerulopathy (6 percent).
Positive family history
• Thin basement membrane nephropathy
• Hereditary nephritis (Alport syndrome)
• Focal segmental glomerulosclerosis are
examples:
LABORATORY TESTING IN PATIENTS WITH SUSPECTED GLOMERULAR DISEASE
• serum creatinine• serum albumin• complete blood count• Estimation of protein excretion in patients
with proteinuria• serologic testing for disorders that cause
glomerular disease• measurement of serum complement levels
membranous nephropathy
• The value of hepatitis B virus
• Hepatitis C virus (HCV) infection
• Congenital and secondary syphilis
• HIV infection (FSGS)
• Chronic bacterial
– Endocarditis
– Shunt nephritis
– Abscesses
• Fungal
• Parasitic infections can cause
SUMMARY AND RECOMMENDATIONS
• There are many causes of glomerular disease,
• the characteristics of the urine sediment,
• The degree of proteinuria
• Patient’s age often
• Two different urinary patterns are seen:
– Nephrotic
– Nephritic.
• The nephrotic pattern
= proteinuria > 3.5 g/day
• Lipiduria
The nephritic
• Red cells
• White blood cells
• Red cell or mixed cellular casts
• Acanthocytes
• Some patients have the concurrent presence of two
glomerular diseases,
Approaching a patient
• Urinalysis
• Estimated glomerular filtration rate
• Patient age help to identify
• Race
• Family history
• Renal biopsy
Standard laboratory testing
• Serum creatinine
• Serum albumin
• Protein excretion in patients with proteinuria
• Serologic testing for disorders that cause
glomerular disease
• Serologic tests – Lupus– Amyloidosis– Hepatitis B (HBV) – Hepatitis C (HCV) – HIV infection– ANA– Anti-dsDNA– C3– C4
• Primary amyloidosis and light chain
deposition disease
– Serum and urine immunofixation
– Serum free light chain ratio analysis
Glomerulonephritis
Nephritic urineRBCs, RBC casts
Low – mod proteinuria
Nephrotic urineNo casts, few RBCsHeavy proteinuria
•Diabetes
•Amyloid
•Membranous Nephropathy
•FSGS
•Minimal Change Disease
•Sometimes MPGN
C3, C4
Low Normal
•Postinfectious
•Lupus nephritis
•MPGN (often Hep C)
progress fast progress slow
ANCA)+( ANCA)-(
•Vasculitis
•Wegener’s
•MPA
•Churg-Strauss
•IgA nepropathy
•Anti-GBM/Goodpasture’s