Clinical Aspects of Glomerular Diseases

Question 1

Nephritic Syndrome:

what does the pt present with clinically?

what do labs show?

Answer 1

Nephritic syndrome is typically an inflammatory process that causes immune-mediated damage

Pt. presents with hematuria, oliguria, HTN

Labs: UA (+) blood, RBC casts, urine protein <3g/ day

Question 2

Nephrotic Syndrome:

damage to the basement membrane causes:

proteinuria level:

____plasma albumin

___ capillary oncotic pressure

and ____ in Antithrombin III, Proteins S and C

Clinical findings:

Answer 2

Nephrotic Syndrome:

damage to the basement membrane causes:

proteinuria level: >3g/d

decreased plasma albumin- hypoalbuminemia

decreased capillary oncotic pressure- edema

and loss in Antithrombin III, Proteins S and C= hypercoaguability

Question 3

what is the urine protein/creatinine ratio in nephrotic syndrome?

what type of casts would we expect to see in UA? why?

Answer 3

protein/creatinine ratio >3.5

we would expect to see fatty casts on UA due to high cholesterol burden in plasma (300-400 mg/dL) which can leak through the damaged epithelium

Question 4

Clinical presentation: edema (periphral, periorbital) massive proteinuria

1. onset
2. etiology:
3. most common cause of nephrotic syndrome in ____
4. associated with:

Answer 4

Minimal Change Disease

1. onset- sudden
2. etiology: idiopathic
3. most common cause of nephrotic syndrome in kids (<10 yo)
4. associated with:
   
   1. medications: lithium, NSAIDS
   2. Lymphomas

Question 5

An African American adult presents with hematuria and high nitrogen blood levels. You look back at his charts and see that his proteinuria levels have gradually increased.

what is the diagnosis?

would this patient respond to steroids?

Answer 5

Nephrotic Syndrome- Focal Segmental Glomerulosclerosis

this patient would not respond to steroid treatment (this is one way you can distinguish it from Minimal Change)

Question 6

FSGS is also associated with what secondary causes?

Answer 6

HIV

sickle cell

heroin use

Question 7

A 50 yo caucasian male presents with proteinuria >3g/day

on biopsy you see the attached image.

What is the antibody associated with this type of lesion?

where is the lesion located?

what should your work up include?

what is your prognosis?

Answer 7

Nephrotic Syndrome: Membranous glomerulonephrpathy

antibodies against the Phopholipase A2 receptor

immune complex deposition in situ on the subepithelial aspect of the BM

work up should include tests for SLE, RA and hepatitis serologies (looking for auto antibodies?) and

**age appropriate cancer screenings**

prognosis: “rule of thirds” 1/3 remission, 1/3 persistent proteinuria with stable renal function, 1/3 progress to ESRD

Question 8

Nephrotic Syndrome- Diabetic Glomerulosclerosis

What would we expect to see on a biopsy?

How do we treat?

Answer 8

Nephrotic Syndrome- Diabetic Glomerulosclerosis

Nodular sclerosis (kimmelsteil-Wilson nodules)

treat by:

glycemic control

BP with ACEI/ARB

intensive cholesterol control

Question 9

Clinical Presentation:

Hematuria

Oliguria

HTN

Labs:

UA: (+) blood, RBC casts

Urine Protein <3g/day

Answer 9

Nephritic Disease

Question 10

A patient comes in complaining of episodes of hematuria. He has a history of URI.

He has normal C3 and C4 blood levels and microscopic hematuria

what is the diagnosis?

Answer 10

A patient comes in complaining of episodes of hematuria. He has a history of URI (viral infection)

He has normal C3 and C4 blood levels and microscopic hematuria

what is the diagnosis?

Nephritic Syndrome IgA Nephropathy

Question 11

an 8 yo male comes into the office with lower extremitiy bruising (palpable purpura), bloody diarrhea andd painful joints.

Dx:

what other clinical findings might we have?

Treatment:

Answer 11

an 8 yo male comes into the office with lower extremitiy bruising (palpable purpura), bloody diarrhea and painful joints.

Dx: Henoch-Scholein Purpura

what other clinical findings might we have? vomitin, abdominal pain, hematuria and hypertension

Treatment: supportive

Question 12

A 12 yo female presents with hematuria. Hx is significant for strep throat.

Dx:

What lab findings distinguish this from IgA Nephropathy?

Prognosis:

Answer 12

A 12 yo female presents with hematuria. Hx is significant for strep throat. (Group A streptococcal infection)

Dx: Post-infectious GN

What lab findings distinguish this from IgA Nephropathy?

LOW C3 and LOW C4

Prognosis: complete recovery

Question 13

Pt labs report low C3 and normal C4

and the image below

Dx:

Hallmarks of this pathology:

Answer 13

Membranoproliferative Glomerulonephritis

1. 2 types:
   
   1. dense deposit disease (excessive activation of the complement pathway)
   2. immune complex localization

membranoproliferative glomerulonephritis has a characteristic double contour formation

Question 14

A patient presents with Hx of sinus infections, (+) hematuria, (+) RBC casts, and sudden onset of HTN.

Dx based on:

prognosis:

What might kill this patient if we miss it?

Answer 14

A patient presents with , (+) hematuria, , and sudden onset of HTN.

Anti-GBM/Goodpasture’s Syndrome

Dx based on: Anti-GBM antibodies and biopsy

prognosis: promptness of dx and treatment and degree of kidney involvement (Cr>5 dialysis is required and progosis is poor)

If there is lung involvement its Goodpasture’s Syndrome

70% of patients with goodpasture’s syndrome will have pulmonary hemorrhage. This is an emergncy and must be treated with plasmapheresis

Question 15

Pt. presents with non specific symptoms of nephritic syndrome but mentions that he has a history of sinus infections:

Dx:

What type of antibody is present?

Answer 15

Granulomatosis with Polyangiitis

(Wegener’s)

ANCA-C is present

Question 16

Pt. presents with non specific symptoms of nephritic syndrome but you notice some skin issues as well.

Dx:

What type of antibody is present?

Answer 16

Microscopic Polyangiitis

(dermato-pulmonary-renal syndrome)

P-ANCA