Lecture 59 - 60: glomerular path (nephritic and nephrotic syndromes)

Question 1

what is the primary indication for renal/glomerular bx

Answer 1

Persistent hematuria or proteinuria

Question 2

in regards to distribution of renal path, define the following:

• diffuse
• focal
• global
• segmental

Answer 2

diffuse: all glomuerli are affected
focal: only some glomeruli are affected (therefore leading to sampling errors)

Global - the entirety of a single glomerulus is affected

Segmental - only part of a glomerulus is affected

Question 3

what are the hallmark symptoms/labs of Nephritic Syndromes

name 4 disease that can be classified as nephritic syndromes:

Answer 3

Hematuria, RBC casts, azotemia, HTN, Mild Edema

Non nephrotic proteinuria (s

Question 4

Post strep GN: 
LM
IF
EM
Labs 

what is the pathognomonic finding

Answer 4

LM: Hypercellulary Glom
IF: Lump Bumpy, granular
EM: Subepithelial humps (Pathog)
Labs: ASO titers positive, Decreased Complement (C3, C4)

Question 5

RPGN – what different disease can cause this process? how are they differentiated

what is the classic Light microscopy finding

Answer 5

RPGN – can be cuased by Goodpastures, SLE, Granulomatous polyangitis (Wegner’s)

differniated by clinical hx, labs and IF

LM: Crescentic

Question 6

Immunofluorescent findings for:
Goodpastures
SLE
Granulomatous Polyangitis

what other lab study is done for Granulomatous polyangitis

Answer 6

Goodpasture’s – Smooth (anti- GBM all along the GMB)

SLE (and PSGN) - Lumpy bumpy, granular

Granulomatous polyangitis – Pauci - Immune (c ANCA positive)

Question 7

Classic findings for Nephrotic syndrome:

Answer 7

Massive proteinuria (> 3.5 g/day)
Pitting edema
also HLD and Lipiduria

Question 8

according to Pathoma what are the three classes of diseases that cause nephrotic syndrome; what are the diseases of each class?

Answer 8

1) Disease of the podocytes: MInimal change disease, FSGS
2) IC Deposition: Membranous Nephropathy, Membranoproliferative disorder (which can also cause nephritic syndrome)
3) Systemic Diseases: DM and Amyloidosis

Question 9

Minimal Change disease -- 
who gets this disease? 
what is the cause?
how do you treat it? 
LM, IF, EM:

Answer 9

Most common Nephrotic disease of Peds
Idiopathic

Responsive to steroids

LM: Normal; IF: Negative
EM: Effacement of the podocytes

Question 10

FSGS -- 
Causes: 
what population commonly at risk 
Is this responsive to steroids? 
LM: 
EM:

Answer 10

idiopathic, HIV, SLE 
At Risk: HIV, heroin users 
Not responsive to steroids 
LM: Focal, segmental damage 
EM: Effacement of the podocytes

Question 11

Membranous Nephropathy -- 
where is the IC deposition? 
Cause: 
LM
EM
IF: 

Treatmnet?

Answer 11

IC Deposition of the Subepithelium

cause: Idiopathic (85%), HBV, HCV, SLE, Tumors, Drugs (15%)

LM: GBM thickening
EM: Spike and Dome
IF: Granular Appearance (random insertion of ICs)

Tx: 30% responsive to steroids

Question 12

Membranoproliferative GN

What are the two types? what are the associated etiologies? where are the IC depositions?

Answer 12

Type 1 - a/w HBV and HCV
Subepithelial IC deposits

Type 2 - a/w C3 nephritic factor; therefore increased complemet.
Intramembranous IC deposits

Question 13

Pathognomonic histological finding of DM nephropathy?

Answer 13

Kimmelstein Wilson Nodules

Question 14

What are the three asymptomatic hematurias/proteinurias?

Answer 14

IgA nephropathy
Alport syndrome
Thin Membrane disease

Question 15

igA Nephropathy –

very specific clinical course
UA;
LM:
IF:

Answer 15

clinical course: Episodic hematuria; esp following mucosal inflammation (even further IgA production)

UA; RBC casts
LM: mesangial proliferation
IF: Immune deposits in the mesangium

Question 16

Alport’s Syndrome

Genetic etiology:
Presentation:
Histo:

Answer 16

Congenital defect (X linked) to Collagen IV

Presentation: Hematuria, Hearing deficits, occuluar disturbance (blood pee, can’t see, cant hearing a buzzing bee)