Diseases

Question 1

Name nephrotic syndromes

Answer 1

MCD, FSGC, Membranous nephropathy, Membranoproliferative, Diabetes and systemic amyloidosis

Question 2

Explain MCD

Answer 2

1. Idiopathic, as are most nephrotic syndromes
2. Protinuria, peritibial and periorbital edema is seen
3. Effacement of foot processes of podocytes on EM
4. Normal findings on light microscopy
5. Excellant response to steroids, however resolves on its own

Question 3

Explain FSGS

Answer 3

1. May progress from MCD
2. Doesn’t respond to steroids, may progress to renal failure
3. Sclerosis can be seen in EM
4. No immune complex deposition, effacement of foot processes

Question 4

Explain membranous nephropathy

Answer 4

1. Subepithelial humps, dome and spike appearence
2. Granular immune complex deposition
3. Thickened basement membrane
4. Poor response to steroids

Question 5

Explain membranoproliferative nephropathy

Answer 5

Type: 1 subendothelial deposits, Type 2: Intramembranous deposit.

1. Most common nephrotic syndrome in lupus patients, remember the most common cause of death in lupus patients is renal failure
2. Granular immune complex deposition

Question 6

Diabetes mellitus

Answer 6

Non enzymatic glycosylation of basement membrane of the renal vasculature leads to hyaline arteriosclerosis, leading to increase in GFR, ACE inhibitors help with reducing this nephrotic syndrome
Kimmelstein-Wilson nodule are pathagnomonic

Question 7

Systemic amyloidosis

Answer 7

Kidneys are the first organ that is affected in systemic amyloidosis, characterized by apple green bifringence under polarized light in congo red staining

Question 8

State nephritic syndrome

Answer 8

1. PSGN
2. RPGN
3. Goodpasteur
4. Diffuse proliferative glomerulonephritis
5. Berger disease
6. Alport syndrome
7. Pauci immune group of diseases

Question 9

PSGN

Answer 9

Post strep infection, either strep throat or cellulitis, cola colored urine, self limiting, on EM we see hypercellular glomeruli
Subepitherlial humps

Question 10

RPGN

Answer 10

Crescents in Bowman’s capsule, nephritis syndrome that progresses to renal failure in months, many diseases (such as Goodpasteur, negative pauci immune cause this)

Question 11

Goodpasteur

Answer 11

Autoimmune antibodies to basement membrane, linear IgG deposits, patients presents with hemoptysis and hematouria

Question 12

Diffuse proliferative glomerulonephritis

Answer 12

Subendothelial humps, most common nephritis syndrome in lupus, granular IgG deposit are seen on IF,

Question 13

Berger disease

Answer 13

IgA immune complexes seen on glomeruli (all the rest of the diseases we talked about had IgG deposits), usually after a mucosal infection like gastroenteritis (since that is where we make IgA), may progress to renal failure

Question 14

Pauci immune disease that affect the kidneys

Answer 14

Diagnostic workup would show nothing on IF, these diseases are Wagner’s (nasal, lungs and kidneys involvement, pANCA), microscopic polyangitis (cANCA) and eosinophilic granulomatous polyangitis (asthma, granulomas, eosinophilia, pANCA)

Question 15

Alport syndrome

Answer 15

Inheritied defect in type IV collagen, X linked,classic triad of hematuria, sensorineural hearing loss and occular disturbances