Ch 16: Kidney- Glomerular Diseases (Part 2) and Vascular Disease Flashcards Preview

Pathology Unit 5 > Ch 16: Kidney- Glomerular Diseases (Part 2) and Vascular Disease > Flashcards

Flashcards in Ch 16: Kidney- Glomerular Diseases (Part 2) and Vascular Disease Deck (17)
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Presents as isolated hematuria, sensory hearing loss, and ocular disturbance. 

What is the inherited defect? 

What happens to the GBM?

When is hematuria present?

What develops later in the course of the disease and by age 40-50 years?

Alport Syndrome (aka Hereditary nephritis)

Type IV Collagen

Splitting, thinning

Early in life for males with X-linked disease, both sexes with AR disease

Proteinuria, progressive renal failure, HTN, end stage renal disease


How can you remember the features of nephritic syndrome?


= Proteinuria, Hematuria, Azotemia, RBC casts, Oliguria, Anti-strep titres (if post-strep), and Hypertension

And also DECREASED GFR due to glomerular inflammation: infiltration by leukocytes, hyperplasia of glomerular cells, necrosis


Which group and hemolytic class of microbe most likely causes postinfectious GN?

What is the usual precipitating infection called?

What is the mechanism of injury causing glomerulonephritis (GN)? How does this look on EM/where is it located?

Group A, Beta-hemolytic streptococci 

impetigo/pyoderma or pharyngitis

Immune complex deposition looks like subepithelial & subendothelial dense deposits (humps) and has a granular (IgG/C3b) IF


How do immune complexes cause inflammation in postinfectious GN?

What causes hypercellularity?

Because you're brilliant and also good looking, you know that when you order an extra panel looking at immune fxn you'll see a significant decrease in this immune cascade protein?

Immune complexes deposit in glomeruli and initiate inflammation by activiting COMPLEMENT and other proinflammatory mediators. 

Proinflammatory mediators activate neutrophils and monocytes which in turn stimulate mesangial and endothelial cell proliferation. (hypercellularity - acute diffuse proliferative GN)



What are the structural features on EM of postinfectious GN?

Subepithelial and subendothelial dense deposits shaped like humps


Which disease is also known as dense deposit disease?

Type II Membranoproliferative GN


What are the 2 types of Membranoproliferative GN and how can you tell them apart?

Both have THICK GBM on H&E: tram-track appearance

think of them as chronic IC diseases

Granular IF due to IC deposition

Type I: Subendothelial deposits (HBV/HCV association)

Type II: Intramembranous deposits (autoantibody called C3 nephritic factor assocation)


What is the most common complication of SLE?


Characterized by widespread involvement of glomeruli and diffuse proliferation of mesangial and endothelial cells and even of epithelial cells. Depositis of IC are present on both sides of the GBM, in the mesangial areas, and inside the capillary loops. 

Wire loop lesions = thickened BM of glomeruli

What is this called?


Diffuse proliferative GN of SLE


Immune complexes of IgA are deposited in the mesangium. They then activate the complement cascade through the alternative pathway. 

Presents as episodic gross or microscopic hematura, with RBC casts, and proteinuria usually follows mucosal infections (gastroenteritis, respiratory tract)

Staining for IgA and C3 on renal biopsy

20% of pts develop renal failure after 10 years

Most often in young men (15-30 peak age of dx)


IgA nephropathy (Berger Disease)


Name the two Anti-GBM antibody diseases.

How are they different from each other?

1) Anti-GBM glomerulonephritis

2) Goodpasture Syndrome

Both have circulating anti-GMB antibodies against type IV collagen in the GBM. 

If lungs and kidneys are both involved = Goodpasture Syndrome


Describe the lung involvement of Goodpasture syndrome. 

anti-GBM antibody against Type IV Collagen is also expressed on pulmonary alveolar capillary basement membranes

These pts would have pulmonary hemorrhages and hemoptysis


These pathological hallmarks are characteristic of which glomerluopathy?

diffuse linear GBM immunostaining for IgG

90% of pts have glomerular crescents (crescentric glomerulonephritis aka acute renal failure) involving over 50% of glomeruli

How are crescents formed?

Is crescentric glomerulonephritis exclusive of the above glomerulopathy?




Goodpasture's syndrome

Crescents are formed from the rupture of GBM and extravasation of blood and inflammatory cells into the urinary space, these structures are composed of proliferating epithelial cells and macrophages

NO, other diseases such as Wegener's granulomatosis or polyarteristis nodosa can have crescents


Most common childhood vasculitis

Caused by vascular localization of IC containing IgA predominantly

Glomerular lesion is identical to that of IgA nephropathy

Other signs/symptoms: diffuse arthralgias, skin rash, violaceous maculopapular rash on lower torso (palpable purpura on buttocks and legs), oliguria, hematuria, usually occurs following an upper Resp tract infection

Henoch-Schonlein purpura (IgA vasculitis)!!!


SPOILER ALERT: Downton Abbey, if you plan on watching this show...avoid this question!!!!!!

You're an avid fan of Downton Abbey, What did Sybil Branson die of? 



What triad characterizes preeclampsia?

What is the difference b/w preeclampsia and eclampsia?

Hypertension, proteinuria, and edema complicating 3rd trimester of pregnancy

Eclampsia is when the above features are complicated by convulsions 


Name 3 diseases that could have rapidly progressive crescentric glomerulonephritis?

1. Goodpasture's Syndrome (5% of all these cases)

2. Wegener's Granulomatosis

3. Microscopic polyarteritis 


Association Station!

1) Nodular glomerulosclerosis aka?

2) Nonenzymtic glycosylation of GBM will result in?

3) Hyperfiltration does what?

4) Microalbuminuria is a sign that you need to give which mediciation?

Dx: Diabetic glomerulopathy

1) Kimmelstiel-Wilson nodules/disease

2) Increased vessel, tubular cell permeability to proteins, hyaline arteriolosclerosis, osmotic damage, increased GFR >> 3) hyperfiltration damage to mesangium

4) ACE Inhibitor time!! Slow progression of diabetic glomerulopathy and retinopathy