Robbins Basic Pathology: Nephritic Syndrome

Question 1

What is nephritic syndrome?

Answer 1

A complex of symptoms characterized by inflammatory infiltrate of the glomerulus followed by hematuria and hemodynamic changes.

Question 2

What are the 4 cardinal signs of nephritic syndrome?

Answer 2

(1) Hematuria with dysmorphic red cells
(2) Oliguria due to decreased GFR
(3) Hypertension due to salt retention
(4) Azotemia due to decreased GFR

Question 3

What causes post-infectious GN?

Answer 3

(1) streptococcal infections
(2) pneumococcal infections
(3) staphylococcal infections
(4) viral infections (measles, mumps, chickenpox, HBV/HCV)

Question 4

What is the typical presentation of post-infectious glomerulonephritis?

Answer 4

GN developing about 1-4 weeks after an infection.

(1) hematuria
(2) oliguria
(3) hypertension
(4) Azotemia

Question 5

What are the characteristics of post infectious GN under light microscopy?

Answer 5

(1) uniform increase in glomerular cellularity (increased endothelial, mesangial, neutrophils, and monocytes)
(2) occasional necrosis and crescentic inflammation

Question 6

What are the characteristics of post infectious GN under electron microscopy?

Answer 6

Sub-epithelial deposits (Humps). Although, occasionaly intramembranous, sub-endothelial, and mesangial deposits may be found.

Question 7

What are the immunoflourescent characteristics of post infectious GN?

Answer 7

Scattered granular deposits of IgG and complement.

Question 8

What is the pathogenesis of post infectious GN?

Answer 8

Immune complex deposition in the glomerulus activates complement and recruits leukocytes which damage the glomerulus.

Question 9

What are the signs of post-infectious GN?

Answer 9

(1) hematuria (grossly visible smokey brown urine)
(2) oliguria (mild)
(3) azotemia (mild)
(4) Hypertension (mild)
(5) proteinuria (<3.5g/dL)
(6) Hypocomplementemia

Question 10

What are the symptoms of post infectious GN?

Answer 10

Malaise
nausea
smokey brown urine

Question 11

How is post infectious GN diagnosed?

Answer 11

An abrupt onset of GN 1-4 weeks after infection should be the first clue.

Question 12

What is the prognosis for post-infectious GN?

Answer 12

(1) most children recover

(2) 15-50% of adults will progress to end stage renal disease.

Question 13

What is IgA nephropathy?

Answer 13

A nephritic disease characterized by recurrent bouts of hematuria following non-specific URI. Also known as Berger’s disease.

Question 14

What is the epidemiology of IgA nephropathy?

Answer 14

IgA nephropathy is the most common nephropathy and typically affects children and young adults. It is one of the most common causes of recurrent hematuria.

Question 15

What is the pathogenesis of IgA nephropathy?

Answer 15

After a non-specific URI excessive amounts of abnormal IgA are often produced. these abnormal IgAs may aggregate and deposit in the mesangium of the glomerulus. This stimulate inflammation that leads to IgA nephropathy.

Question 16

What are the microscopic findings associated with IgA nephropathy?

Answer 16

(1) highly variable focal proliferative glomerulonephropathy.
(2) mesangial widening
(3) Dense mesangial deposits (electron microscopy)

Question 17

What are the immunoflourescent findings associated with IgA nephropathy?

Answer 17

Mesangial IgA with depostion of C3 and properdin. Also lesser amounts of IgG and IgM may be found.

Question 18

What are the signs and symptoms of IgA nephropathy?

Answer 18

Recurrent hematuria presents in the vast majority of IgA nephropathy cases and 5-10% can progress to complete nephritic syndrome.

Question 19

What is the treatment for IgA nephropathy?

Answer 19

Corticosteroids can reduce the proteinuria. If HTN is present it must be treated.

Question 20

What is the prognosis for IgA nephropathy?

Answer 20

IgA nephropathy experiences a slow progression, however, 40-50% progress to chronic renal failure.

Question 21

What is rapidly progressive crescentic glomerulonphritis?

Answer 21

A variation of the nephritic syndrome which includes the crescentic proliferation of parietal epithelial cells and leukocytes.

Question 22

What is the pathogenesis of type I CrGN?

Answer 22

Anti-GBM antibodies stimulate inflammation and causes crescentic inflammation.

(1) Idiopathic
(2) Goodpasture’s syndrome

Question 23

What is the pathogenesis of type II CrGN?

Answer 23

Immune complex deposition stimulates crescentic inflammation.

(1) post-infectious
(2) SLE
(3) Henroch-Schonlein/IgA nephropathy.
(4) idiopathic.

Question 24

What is the pathogenesis of type III CrGN?

Answer 24

Anti-Neutrophil cytoplasmic antibodies stimulate crescentic inflammation.

(1) Wegener’s granulomatosis
(2) Microscopic angiitis.

Question 25

What is Goodpastures syndrome and what separates it from idiopathic type I CrGN?

Answer 25

Goodpastures syndrome is caused by anti-GBM antibodies that target the basement membranes in the glomerulus and the lungs. In the glomerulus this stimulates crescentic inflammation. If only the glomerulus is involved this is called idiopathic CrGN.

Question 26

What is the gross pathology associated with Goodpastures syndrome?

Answer 26

In Goodpastures syndrome the kidney is found to be pale and have petechial hemorrhages over the cortical surface.

Question 27

What is the microscopic and immunoflourescent pathology associated with Goodpastures syndrome?

Answer 27

Segmental necrosis of the glomerulus with GBM breaks and linear staining of the GBM (IgG and C3)

Question 28

How is type I CrGN (Goodpastures syndrome) treated?

Answer 28

Plasmapharesis is an effective way of removing the anti-GBM antibodies from the serum.

Question 29

What is the microscopic pathology associated with type II CrGN?

Answer 29

(1) segmental necrosis (2) GBM breaks (3) Crescent formation

Question 30

What are the immunoflourescent findings associated with type II CrGN?

Answer 30

Lumpy/bumpy granular GBM staining due to IgG and C3 complex deposition within the GBM.

Question 31

What are the microscopic and immunoflourescent findings associated with Type III CrGN?

Answer 31

(1) lack of immunoflourescense (2) Crescentic inflammation (3) GBM breaks (4) segmental necrosis.

Question 32

What is chronic glomerulonephritis?

Answer 32

The progression to end-stage renal failure seen in many nephritic and nephrotic syndromes.

Question 33

What is the epidemiology of chronic GN?

Answer 33

30-50% of all long term hemodialysis and kidney transplant patients develop chronic GN.

Question 34

What is the pathogenesis of chronic GN?

Answer 34

prolonged inflammation of the glomeruli leads to scarring, obliteration, and interstitial fibrosis.

Question 35

What is the gross pathology of chronic GN?

Answer 35

The kidneys are contracted with a reddish brown color and petechial hemorrhages over the cortical surfaces.

Question 36

What are the microscopic findings associated with chronic GN?

Answer 36

Scarring of the glomeruli, potentially complete obliteration with interstitial fibrosis and infiltrate.

Question 37

What are the signs of chronic GN?

Answer 37

(1) proteinuria that lessens with progression (2) HTN (3) Azotemia (4) microscopic hematuria

Question 38

How is chronic GN treated?

Answer 38

(1) renal dialysis | (2) kidney transplant

Question 39

What is the prognosis for Chronic GN?

Answer 39

The prognosis is poor. Without treatment disease progresses to uremia and death. However, renal transplant and dialysis can considerably prolong life.