B71 nephritic syndrome

Question 1

What are the causes of nephritic syndrome?

Answer 1

1. IgA nephropathy, aka Berger disease
2. RPGN
3. Acute postinfectious GN
4. Goodpasture syndrome
5. SLE - immune complex mediated
6. Wegener’s granulomatosis
7. Hemolytic uremic syndrome
8. Henoch-schonlein purpura
9. Infective endocarditis
10. Alport syndrome - Mutation of Type 4 collagen, main component of GBM, nephrosis leading to ESRD

Question 2

What are the clinical symptoms of nephritic syndrome?

Answer 2

Hematuria with dysmorphic RBCs and RBC casts

Proteinuria less than 3.5 g/day

Oliguria/anuria

Azotemia

Mild hypertension

Mild edema, especially periorbital edema

Hypercellular, inflamed glomeruli on biopsy

Question 4

Describe the pathogenesis of IgA nephropathy

Answer 4

IgA nephropathy, Berger disease

After a non-specific upper respiratory tract infection, and in patients where there is some disorder of either abnormally high IgA prodution or slow IgA clearance

Many patients have excessive IgA production by the marrow.

Abnormal IgA glycosylation increases half life

Deposition of IgA in mesangium, complement activation , recruitment of neutrophils, nephritic syndrome.

Question 5

What is the clinical presentation of Berger disease?

Answer 5

Presents in children and young adults as recurrent bouts of nephritis syndrome, which after accumulated events progresses to renal failure over about 20 years.

The nephritis occurs within days of an upper resp. infection as opposed to the post=strep GN, which occurs 2-3 weeks later.

Question 7

Describe the pathogenesis of acute post infectious GN

Answer 7

Immune complex formation in the circulation, deposit into the glomeruli.

Deposit in the subendothelial layer. Produces subendothelial humps on EM.

Stimulate mesangial cell proliferation.

Hypercellular, inflammed glomeruli.

Question 8

Describe the presentation and clinical course of post-infectious GN

Answer 8

2-3 weeks after group A strep or sometimes S. aureus, S. pyogenes

Presents as hematuria, oliguria, hypertension, and periorbital edema

Most often in children but can occur in adults.

Treatment is supportive

1% of children progress to RPGN

25% of adults progress to RPGN.