glomerulonephritis and PKD Flashcards
(58 cards)
1
Q
define glomerulonephritis
A
- diseases that present in the nephritic spectrum and usually signifies an inflammatory process involving the glomeruli, causing renal dysfunction
2
Q
What are the 4 main components of the glomerulus
A
- podocytes
- glomerular basement membrane
- capillary endothelium
- mesangium
3
Q
damage to podocytes leads to
A
increased permeability and allows substances to get into the urine
4
Q
glomerulonephritis is characterized by what two things
A
- intraglomerular inflammatory process
- renal dysfunction
5
Q
List the 3 conditions in the nephritic spectrum
A
- asymptomatic glomerular hematuria
- nephritic syndrome
- rapidly progressive glomerulonephritis
6
Q
“Glomerular” hematuria, dysmorphic RBCs, RBC casts and “cola-colored” urine are all consistent with what condition
A
Nephritic syndrome
7
Q
What conditions are associated with RBC casts?
A
- glomerulonephritis
- vasculitis
8
Q
Nephritic syndrome usually has how much protein in the urine
A
- “Subnephrotic” proteinuria
- < 3.0 g/ 24 hr
9
Q
clinical presentation
- cola colored urine
- edema
- face and eyes in am
- feet and ankles in pm
- HTN
- decreased GFR
- oliguria
A
Nephritic syndrome
10
Q
which condition is caused from a severe injury to the glomerular wall and leads to a crescent formation (crescentic GN)
A
- rapidly progressive glomerulonephritis (RPGN)
- most severe and clinically urgent of nephritic spectrum
11
Q
What is the one clinical sign that is common in all conditions in the nephritic spectrum
A
hematuria
12
Q
distinguish extraglomerular hematuria in terms of
- color
- clots
- proteinuria
- RBC morphology
- RBC casts
A
- color
- red or pink
- clots
- may be present
- proteinuria
- < 500 mg/day
- RBC morphology
- normal
- RBC casts
- absent
13
Q
distinguish glomerular hematuria in terms of
- color
- clots
- proteinuria
- RBC morphology
- RBC casts
A
- color
- red, smoky, brown or coca-cola colored
- clots
- absent
- proteinuria
- may be >500mg/day
- RBC morphology
- some RBCs are dysmorphic
- RBC casts
- may be present
14
Q
What are the three primary disease causes of glomerulonephritis
A
- IgA nephropathy
- poststreptococcal GN
- anti-GBM GN
15
Q
What are the three secondary disease causes of glomerulonephritis
A
- Lupus nephritis
- Henoch-Schonlein purpura
- Renal vascular disease
16
Q
IgA nephropathy (Bergers disease) affects what patient populations
A
- 2nd or 3rd decades of life
- male
17
Q
what causes IgA nephropathy (Bergers disease)
A
- idiopathic
- immune complex mediated GN
- IgA deposition in the glomerular mesangium -> inflammatory process
18
Q
clinical presentation
- episode of gross hematuria
- often follows URI
- urine becomes cola-colored 1-2 days after illness onset
A
- IgA nephropathy (Bergers disease)
- can present anywhere along the nephritic spectrum
19
Q
when should patients with IgA nephropathy (Bergers disease) be treated? what is the tx?
A
- treat pt’s with HTN, proteinuria > 1g/d, decreased GFR
- ACE-I or ARB
- immunosuppressive meds
- 1/3 have spontaneous remission
20
Q
poststreptococcal GN affects what patient populations
A
- bimodal
- > 60 yo
- children ages 5-12 yo
21
Q
describe etiology of poststreptococcal GN
A
- induced by group A strep infection
- deposition of streptococcal nephritogenic antigens within the glomerulus -> triggers complement activation and inflammation
22
Q
clinical presentation
- varies across nephritic spectrum
- occurs 1-3 weeks after infection
- pharyngitis or impetigo
- elevated antistreptolysin O titers
A
poststreptococcal GN
23
Q
tx of poststreptococcal GN
A
- supportive
- children more likely to fully recover
24
Q
Describe Anti-GBM antibody disease
A
- autoantibodies directed against an antigen intrinsic to the glomerular basement membrane (GBM)
25
Anti-GBM antibody disease usually affects what patient population
* bimodal
* M: 30s
* M & F: 60-70s
26
Anti-GBM antibody disease can present with what two conditions
* glomerulonephritis alone OR
* **Goodpasture's syndrome**
27
what does a patient with Goodpasture's syndrome present with
* **pulmonary hemorrhage** and
* **glomerulonephritis**
28
How do you test for anti-GBM antibodies
* **kidney biopsy**
29
treatment of anti-GBM antibody disease
plasmapheresis + prednisone + cyclophosphamide
30
explain how SLE causes glomerulonephritis
* Anti-dsDNA antibodies seen in SLE are nephritogenic
31
What is Henoch-Schonlein Purpura
* systemic, small vessel vasculitis
* IgA immune complex deposition
* skin, GI tract, kidneys
32
Henoch-Schonlein Purpura usually affects what patient population
* children
* male
33
Henoch-Schonlein Purpura is often preceded by what
* URI often precedes by 1-3 weeks
* i.e. streptococcal infections
34
list the tetrad of clinical manifestations of Henoch-Schonlein Purpura
1. palpable purpura
2. arthritis/arthalgias
3. abdominal pain
4. renal disease -\> glomerulonephritis
35
tx of Henoch-Schonlein Purpura
* supportive
* +/- immunosuppressive medications and plasmapheresis
36
**Pauci-immune glomerulonephritis** is caused by the following system **ANCA-associated** small vessel _vasculitides_
1. **Granulomatosis with polyangiitis** (Wegeners)
2. **Microscopic polyangiitis**
3. **Eosinophilic granulomatosis with polyangiitis** (Churg-Strauss syndrome)
37
what are the common characteristics seen in Pauci-immune glomerulonephritis
* M=F
* \> or = 55
* **Absence** of immune deposits
* frequently presents as rapid progressive GN
* 50% have pulmonary disease
38
What is the problem with ANCAs
* they bind to antigens -\> tissue damage
39
Granulomatosis with Polyangiitis presents with what triad of symptoms
1. Glomerulonephritis
2. Upper respiratory tract involvement
3. Lower respiratory tract involvement
40
clinical presentation
* fever, myalgia, arthralgia
* otorrhea, purulent rhinorrhea, nasal ulcers
* cough, hemoptysis, SOB
* microscopic hematuria, proteinuria
Granulomatosis with Polyangiitis
41
Microscopic polyangiitis has a similar presentation to granulomatosis with polyangiitis except
* rarely have significant lung disease
* rarely have destructive sinusitis
42
patients with a hx of what are more susceptible to get Eosinophilic Granulomatosis with Polyangiitis
* asthma
* eosinophilia
43
Eosinophilic Granulomatosis with Polyangiitis develops in what 3 phases
1. prodromal phase
2. eosinophilic phase
3. vasculitic phase
44
Presentation
* segmental **transmural inflammation** of muscular **arteries**
* middle-aged, older adult males
* tendency to **spare the lungs**
* **ANCA is Negative**
Polyarteritis Nodosa
45
Polyarteritis Nodosa is caused by
* idiopathic
* triggered by HBV
46
explain how transmural inflammation causes
* causes luminal narrowing -\> thrombosis -\> ischemia/infarction
* causes weakening of vessel wall -\> microaneurysms -\> hemorrhage
47
clinical presentation
* PNS and dermatolgoic symptoms
* renal manifestations (60%)
* renal **infarctions** -\> proteinuria, hematuria, renal insufficiency
* RBC casts are absent
Polyarteritis Nodosa
* \*\*this is a vasculitis, does not cause GN
48
positive C-ANCA is consistent with
granulomatosis with polyangiitis
49
Treatment for both Pauci-Immune GN and polyarteritis Nodosa
* immunosuppressive
* +/- plasmapheresis
* steroid taper
* immunosuppressive agents
50
What is the most common form of polycystic kidney disease? what is it caused by
* autosomal dominant PKD
* mutations in PKD-1 or PKD-2 gene
51
when does autosomal dominant PKD develop
40s or 50s
52
autosomal dominant PKD causes cysts on bilat kidneys, kidney enlargement and decline in renal function. what are some extrarenal manifestations
* cerebral aneurysms
* cardiac valvular abnormalities
* hepatic, splenic, pancreatic cysts
53
clinical presentation
* large, palpable kidneys on exam
* HTN
* hematuria
* nephrolithiasis
* UTI
* mild proteinuria
autosomal dominant PKD
54
how is autosomal dominant PKD diagnosed
US: initial test and diagnostic
55
If a patient has PKD and HTN, he/she should be treated with
ACE-I or ARB
56
autosomal recessive PKD usually affects what age group? what gene is affected
* infants and children
* mutation in PKHD1 gene
57
autosomal recessive PKD usually affects what two organs
* kidneys
* biliary tract
* see bilateral markedly enlarged kidneys and congenital hepatic fibrosis at birth
58
How is autosomal recessive PKD diagnosed and treated
* US
* supportive
* HTN management
* dialysis, kidney transplant
* possible liver transplant
