Glomerular Disease wiht nephritic and nephrotic syndrom

1. GLOMERULAR DISEASE
Yousaf Khan
Lecturer Renal Dialysis
IPMS-KMU

2. GLOMERULAR DISEASE
 Group of disease
 Affect of glomerular and inflammatory in nature
 Immunologically mediated
 It may be primary or secondary.

3. PRIMARY GLOMERULAR DISEASE
 Minimal change glomerular disease
 Membranous glomerulonephritis
 Membranoproliferative glomerulonphritis
 IgA nephropathy
 Acute diffuseproliferative glomerulonephritis

4. SECONDARY GLOMERULAR NEPHRITIS
Common
 SLE
 Polyarteritisnodosa
 Diabetes mellitus
 Amyloidosis
 Malarial nephropathy
 Uncommon:
 Sarcoidosis
 Rheumatoid arthritis
 Hemolytic uremicsyndorm
 AIDS nephropaty

5. PATHOGENESIS
1. Circulating immune complex deposition:
Antigen + Antibody – deposition in glomeruli – binding with
complement – inflammation – glomerular injury.
 Antigen may be exogenous or endogenous
2. Antibodies directed against antigen on glomerular
capillary membrane:
 Anti – GBM antibody disease – antigen fixed in the GBM e.g
good pasture syndrome
 Antibodies against non GBM antigen

6. NEPHROTIC SYNDROME
Clinical complex characterized by
 Heavy proteinuria (3.5 g/day)
 Hypoalbuminemia
 Edema
 Hypelipidemia
 Hyperlipiduria
Proteinuria:
 daily loss of protein 3.5 gm or more of protein
 Injury to the capillary wall of the glomeruli result – increase
permeability to the plasma protein – allow to – escape from
plasma

7. PATHOGENESIS
Hypoalbuminemia:
 Proteinuria – decrease serum albumin level
Generalized edema:
 Hypoalbuminemia – result decrease colloid osmotic pressure
Hyperlipidemia:
 Hypoalbuminemia triggers increase sysnthesis of all form of
plasma protein including lipoprotein – hyperlipidemia.
Hyperlipiduria:
 Hyperlipoproteinemia – increase permeability results in
hyperliduria

8. ETIOLOGY OF NEPHROTIC SYNDROME
Primary glomerular disease:
 Minimal change nephropathy
 Focal segmental glomerulosclerosis
 Membranous GN
Secondary GN associated with systemic disease:
 Diabetic nephropathy
 Amyloidoisis
 Drugs: penicillamine, gold, mercury, cadmium
 Allergic reaction

9. CLINICAL FEATURES
Edema:
 Upper and lower limb
 Children more obvious on the face
 Intense edema of scrotum or vulva may occur
 Bilateral hydrothorax
 Edema of intestine causes anorexia, diarrhea and vomiting.
Malnutrition:
 Malnutrition may be due to protienuria, frequent infection and
muscle wasting.
Hypercoagulability:
 Hypercoagulability manifest as peripheral arterial or venous
thrombosis renal vain thrombosis and pulmonary embolism

10. INVESTIGATION
 Urine D/R – Proteinuria
 24 hr urinary protien - > 3 g/day
 Serum albumin – less than 3 g/dl and total serum protein < 6
mg/dl
 Low- density lipoprotien is elevated but HDL is usually normal.
 Raised ESR due to increase serum fibrinogen
 Blood sugar for diabetes and antinuclear factor for SLE.
 Serology and renal biopsy.

11. COMPLICATION AND MENAGEMENT
 Protein malnutrition
 Hypercoagulabilty – due to rise in many clotting factors
 Impaired resistance to infection
 Sepsis, blood loss and hpovolemia may lead to acute oliguric
renal failure
Management:
 Diet
 Diuretics
 Hypercholesterlemia
 Hypercoagulability
 Oliguric renal failure

12. MEMBRANOUS GLOMERULONEPHRITIS
 Slowly progressive disease
 Most common between 30 to 50 year
 Characterized by diffuse thickening of GBM
 Cause by deposition of immune complex on the
epithelial side of the GBM.

13. ETIOLOGY
 Primary 85% membranous nephropathy caused by
autoantibodies that cross-react with antigens expressed
by podocytes.
Secondary causes
 Infections (chronic hepatitis B, syphilis, malaria)
 Malignant tumors, particularly carcinoma of the lung and
colon and melanoma
 Systemic lupus erythematosus and other autoimmune
conditions
 Exposure to inorganic salts (gold, mercury)
 Drugs (penicillamine, captopril, nonsteroidal
antiinflammatory agents)

14. Pathogenesis:
 Membranous glomerulonephritis is a form of chronic immune
complex nephritis
Morphology:
Feature of light microscope
 Diffuse thickening of glomerular basement membrane
Feature of electron microscope:
 Apparent thickening of GBM is caused by subepitheial
deposits that are separated from each other by small spike in
GBM matrix.

15. CLINICAL FEATURE OF MEMBRANOUS GN
 Insidious development of nephrotic syndrome.
 In contrast to minimal change disease the proteinuria is non
selective (albumin and globulin both are excreted)
 Usually does not response to corticosteroid therapy
 About 40% lead to renal failure after 2- 20 years
 60% although proteinuria persist yet they do not progress to
renal failure

16. NEPHRITIC SYNDROME
 Inflammatory process causing renal dysfunction
 Over days to week that may or may not resolve.
 More than 50% loss of nephron function
Characterized by
 Hematuria with RBC casts
 Proteinuria (usually non rephrotic range)
 Hypertension
 Edema
 Oliguria
 uremia

17. Glomerual disease with nephritic presentation
 Post- Streptococcal GN
 IgA nephropaty
 Goodpasteur sysndrom
 Polyarteritits nodosa
 Acute interstitial nephritis

18. Investigation:
Urinalysis
 Dysmorphic red cell
 Red cell cast
 Proteinuria
Serum chemistries
Renal biopsy
Treatment:
 Reduction of hypertension
 Salt water restriction
 Diuretics

19. RAPIDLY PROGRESSIVE
GLOMERULONEPHITIS
Yousaf Khan
Lecturer Renal Dialysis
IPMS-KMU

20. RPGN
 Clinical syndrome
 Characterized by loss of renal function
 Laboratory finding – nephritic syndrome – severe oliguria
 About 50% cases are idiopathic while 50% are related to the
systemic disease.
 Histological finding associated with RPGN is the presence of
cresents

21. TYPES OF RPGN
Type I RPGN:
Anti-GBM disease
 Characterized by deposition of IgG and C3 on GBM
 anti-GBM antibodies cross react with pulmonary alveolar
basement membrane –to produce clinical picture of pulmonary
hemorrhage along with renal hemorrhage – good pasture
syndrome.
 Type II RPGN (Immune – complex mediated disease)
 Complication of any of immune complex nephritis such as
poststrptococcal GN, SLE, IgA nephropathy ets
 Type III:
 Characterized by lack of anti GBM antibodies or immune
complex by immunoflurescent and electron microscope.
 In serum antineutrophilic cytoplasmic antibody
 Associated with some systemic vasculitis

22. MORPHOLOGY
 Presence of crescents in most of the glomeruli
 Crescents are formed by proliferation of the parietal epithelial cells of
bowman capsule
 Crescents eventually obliterate the bowman space and compress the
glomeruli resulting oliguria

23. CLINICAL FEATURE
 Like nephritic syndrome
 But more marked oliguria and azotemia
 90% patient required dialysis and transplantation.

24. Thank You