09 Adult Nephrotic Syndrome

8/12/2019 09 Adult Nephrotic Syndrome

1/77

V in e et a V Ba tr aD e p t o f P at h o l o g yG B P a n t H o s p i t a l

Adult Nephrotic Syndromecase based discussion


2/77

Nephrotic syndrome

Proteinuria (>3.5g/day)

Hypoalbuminemia

Hyperlipidemia

Lipiduria

Edema

Bland urine sediment


3/77


4/77

Nephrotic syndromein adultsPrimary

Secondary


5/77

Structural abnormality in

primary nephrotic syndrome

-diffuse podocytopathies

- diffuse simplification or fusion of

foot processes of glomerular

visceral epithelial cells


6/77

CL

End

FP

Ep

US


7/77


8/77


9/77

Common causes of primarynephrotic syndrome in adultsPrimary

- Minimal change disease

- Focal segmental glomerulosclerosis

- Collapsing glomerulopathy

- Membranous nephropathy


10/77

Causes of nephrotic syndrome in adultsSecondary- Membranoproliferative glomerulonephritis

(MPGN)

- Diffuse and nodular diabetic GS

- Amyloidosis

- Systemic Lupus erythematosus

- Monoclonal immunoglobulin deposition dis.

- Fibrillary GN

- Immunotactoid glomerulopathy


11/77

Secondary nephrotic syndromeDrugs

Gold

Antimicrobial agents

Non-steroidal anti-

inflammatory drugs

Penicillamine

Captopril

Tamoxifen

Lithium

Infections

HIV

Hepatitis B and C

Mycoplasma

Syphilis

Malaria

Schistosomiasis

Filariasis

Toxoplasmosis


12/77

Secondary nephrotic syndrome in adultsHematologic and oncologic diseases

Myeloma

Lymphoma (Hodgkins most likely)

Congenital causesAlports syndrome

Congenital NS of the Finnish type

Piersons syndromeNail-patella syndrome

Denys-Drash syndrome


13/77

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 withsignificant proteinuria

serum creatinine was 2.1 mg/dl


14/77


15/77


16/77


17/77


18/77

IgG


19/77


20/77

Final Diagnosis

Minimal change disease


21/77

Case 2

30-year-old woman developed sudden-onsetedema

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


22/77


23/77


24/77

C3


25/77

Final Diagnosis

Focal and segmentalglomerulosclerosis


26/77

FSGS1. Idiopathic or primary FSGS

2. Secondary or adaptive3. Familial

4. Segmental glomerular scarring


27/77

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


28/77


29/77

IDIOPATHIC OR

PRIMARY FSGS

ADAPTIVE OR

SECONDARY FSGS


30/77


31/77

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


32/77

Case 3

32 year old HIV positive male

Developed sudden onset massive

proteinuria to the extent of 12gm/24

hours

No response to treatment


33/77


34/77


35/77


36/77

Albumin


37/77


38/77


39/77

FINAL DIAGNOSIS

COLLAPSINGGLOMERULOPATHY


40/77

Collapsing glomerulopathy


41/77

Case 4

17 year old female presenting with

sudden onset edema

Nephrotic range proteinuria

Urine exam: Significant proteinuria

with few RBCs which were dysmorphic


42/77


43/77


44/77

IgG


45/77

Final Diagnosis

MEMBRANOUSGLOMERULOPATHY


46/77

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

C diti i t d ith


47/77

Conditions associated with


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

C di i i d i h


48/77

Conditions associated with


5.- Miscellaneous

Tumors

Sarcoidosis

Renal allograft

Sickle cell disease

Fanconi's syndrome

Crohn's disease, HUS,alpha-1-antitrypsin deficiency

Guillain-Barre syndrome


49/77


50/77

Case 5

14 yr /F presented with swelling over body for 45days

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


51/77


52/77


53/77


54/77


55/77


56/77

FINAL DIAGNOSIS

MEMBRANOPOLIFERATIVE

GLOMERULONEPHRITIS


57/77

Case 6

62 year old woman presenting with

- anemia,

- generalized body edema

- and massive proteinuria


58/77

HE 10x showing renal

biopsy in low power


59/77


60/77


61/77


62/77


63/77


64/77


65/77

IgG


66/77

IgG


67/77

lambda kappa


68/77


69/77

FINAL DIAGNOSIS


70/77

FINAL DIAGNOSIS

Primary Amyloidosis

involving the glomeruli,

interstitium and blood

vessels


71/77

AA Amyloidstain

C 7


72/77

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


73/77


74/77


75/77


76/77


77/77

Thank you

09 Adult Nephrotic Syndrome

Documents