Vi neet a V Bat ra

Dept of Pat hol ogy

GB Pant Hospi t al
Nephrotic syndrome
Proteinuria (>3.5g/day)
Hypoalbuminemia
Hyperlipidemia
Lipiduria
Edema
Bland urine sediment
Nephrotic syndrome
in adults
Primary
Secondary
Structural abnormality in
primary nephrotic syndrome
-diffuse podocytopathies
- diffuse simplification or fusion of
foot processes of glomerular
visceral epithelial cells
CL
End
FP
Ep
US
Common causes of primary
nephrotic syndrome in adults
Primary
- Minimal change disease
- Focal segmental glomerulosclerosis
- Collapsing glomerulopathy
- Membranous nephropathy
Causes of nephrotic syndrome in adults
Secondary
- Membranoproliferative glomerulonephritis
(MPGN)
- Diffuse and nodular diabetic GS
- Amyloidosis
- Systemic Lupus erythematosus
- Monoclonal immunoglobulin deposition dis.
- Fibrillary GN
- Immunotactoid glomerulopathy
Secondary nephrotic syndrome
Drugs
Gold
Antimicrobial agents
Non-steroidal anti-
inflammatory drugs
Penicillamine
Captopril
Tamoxifen
Lithium
Infections
HIV
Hepatitis B and C
Mycoplasma
Syphilis
Malaria
Schistosomiasis
Filariasis
Toxoplasmosis
Secondary nephrotic syndrome in adults
Hematologic and oncologic diseases
Myeloma
Lymphoma (Hodgkins most likely)
Congenital causes
Alports syndrome
Congenital NS of the Finnish type
Piersons syndrome
Nail-patella syndrome
Denys-Drash syndrome
Case 1
17-year-old woman with unremarkable past
medical history
Developed sudden-onset edema
Physical examination
Obese
3+ pitting edema of lower extremities
Urine exam: inactive sediment with
significant proteinuria
serum creatinine was 2.1 mg/dl
IgG
Final Diagnosis
Minimal change disease
Case 2
30-year-old woman developed sudden-onset
edema
Physical examination
pitting edema of lower extremities
Urine: Inactive sediment with significant
proteinuria, occasional fresh RBCs seen
Serum creatinine was 1.51 mg/dL
24 hour urinary protein levels 5.5gm
C3
Final Diagnosis
Focal and segmental
glomerulosclerosis
FSGS
1. Idiopathic or primary FSGS
2. Secondary or adaptive
3. Familial
4. Segmental glomerular scarring
Differentiating primary FSGS
from secondary FSGS
Primary
Sudden onset
Nephrotic range proteinuria
Generalized body edema
Hypoalbuminemia
No history of prior kidney disease
Normal size glomeruli
No significant parenchymal atrophy
EM: Diffuse effacement of foot
processes
Responds well to treatment
Secondary
Gradual onset
mild proteinuria ( < 2 g/24h)
No edema
No hypoalbuminemia
History of prior kidney / vascular ds
Glomerular hypertrophy
Pre-existing kidney injury
EM: Focal effacement of foot
processes
Partial response to treatment
IDIOPATHIC OR
PRIMARY FSGS
ADAPTIVE OR
SECONDARY FSGS
FOCAL AND SEGMENTAL GLOMERULAR
SCARRING
Secondary to a healed glomerulitis:
IgA nephropathy/ Henoch-Schoenlein purpura
Various vasculitides, early phase
Early idiopathic crescentic glomerulonephritis
Immune complex-mediated diseases
- Lupus nephritis, WHO class III
- Post infectious GN
Case 3
32 year old HIV positive male
Developed sudden onset massive
proteinuria to the extent of 12gm/24
hours
No response to treatment
Albumin
FINAL DIAGNOSIS
COLLAPSING
GLOMERULOPATHY
Collapsing glomerulopathy
Case 4
17 year old female presenting with
sudden onset edema
Nephrotic range proteinuria
Urine exam: Significant proteinuria
with few RBCs which were dysmorphic
IgG
Final Diagnosis
MEMBRANOUS
GLOMERULOPATHY
Conditions associated with secondary
membranous nephropathy
1. Autoimmune Diseases
SLE , RA
Hashimoto's disease, Grave's disease,
MCTD, Sjgren's syndrome,
primary biliary cirrhosis,
bullous pemphigoid, dermatitis
herpetiformis, ankylosing spondylitis,
small bowel enteropathies
Conditions associated with
membranous nephropathy
2.- Infectious or Parasitic Diseases
Hepatitis B and C
Syphilis, Filariasis,
hydatid cyst, schistosomiasis,
malaria, leprosy
3.- Drugs
Au, Hg, Penicillamine, Captopril,
NSAIDs, Hydrochlorothiazide, Hydralazine,
Trimethadione, Chlormethiazole
Conditions associated with
membranous nephropathy
5.- Miscellaneous
Tumors
Sarcoidosis
Renal allograft
Sickle cell disease
Fanconi's syndrome
Crohn's disease, HUS,
alpha-1-antitrypsin deficiency
Guillain-Barre syndrome
Secondary Nephrotic
Syndrome
Case 5
14 yr /F presented with swelling over body for 45
days
Lab values
Urine - inactive sediment with significant proteinuria
Serum protein 3.9, Serum creatinine 0.9,
24 hr urinary protein 4.5gm
Hep B and C & HIV serology negative
ANA negative
C3 levels were 30 mg/dl
FINAL DIAGNOSIS
MEMBRANOPOLIFERATIVE
GLOMERULONEPHRITIS
Case 6
62 year old woman presenting with
- anemia,
- generalized body edema
- and massive proteinuria
HE 10x showing renal
biopsy in low power
IgG
IgG
lambda kappa
FINAL DIAGNOSIS
Primary Amyloidosis
involving the glomeruli,
interstitium and blood
vessels
AA Amyloid
stain
Case 7
55 year old male
Known case of diabetes mellitus for last 12
years
Now presenting with persistent albuminuria
(>400 mg/dl)
Rise in serum creatinine to 2mg/dl
hypertension
Final Diagnosis
Diabetic nephropathy
Thank you