Nephritic glomerular diseases Flashcards
1
Q
in Nephritic glomerular disease is limited to
A
the glomerulus
2
Q
Postinfectious GN is the classic nephritic disease, an have the following clinical picture:
A
1-dark urine,
2-hypertension,
3-and edema developing suddenly 1–2 weeks after strep pharyngitis
3
Q
If not caused by group A beta hemolytic streptococci (Streptococcus pyogenes), it may be caused by
A
-throat or skin infection with Streptococcus pyogenes
4
Q
Poststreptococcal GN occurs in 10–15% of patients with
A
pharyngitis
5
Q
Virtually any infectious agent can cause postinfectious GN, including
A
1-hepatitis B and C,
2-CMV,
3-and chronic staphylococcal infections such as endocarditis
6
Q
The key to diagnosis is an association with
A
infection
7
Q
The best initial test is the
A
antistreptolysin (ASO) or antihyaluronic acid (AHT)
8
Q
Complement levels, particularly C3, are
A
low.
9
Q
Renal biopsy is rarely needed, but if done would show epithelial “………..” on electron microscopy. IgG and C3 will be deposited in the mesangium.
A
humps
10
Q
Treatment of Poststreptococcal GN is
A
supportive (management of fluid overload and hypertension with diuretics). Most cases resolve spontaneously.
11
Q
…………. will eradicate the organism from the pharynx
A
Antibiotics
12
Q
……………… are sometimes used for unusual persistence of proteinuria or renal failure in adults.
A
Glucocorticoids
13
Q
define Goodpasture syndrome (GPS)
A
is an idiopathic renal and lung disease characterized by a unique anti-glomerular basement membrane antibody
14
Q
clinical Presentation includes
A
hematuria and hemoptysis.
15
Q
Aside from the………….. and …………….. , GPS does not affect other sites in the body
A
lungs and kidneys
16
Q
thus an absence of skin or eye findings is a clue to
A
the diagnosis.
17
Q
When there is lung involvement (65%), patients present with
A
1-hemoptysis,
2-cough,
3-shortness of breath.
18
Q
The key to diagnosis of Goodpasture syndrome (GPS) is
A
nephritic-pulmonary syndrome
19
Q
The best initial test for nephritic-pulmonary syndrome is
A
the level of antibasement membrane antibodies to type IV collagen.
20
Q
The single most accurate test is
A
lung or kidney biopsy, which will show linear deposits on immunofluorescence
21
Q
Do lung biopsy, not renal, if there is …………………… involvement.
A
pulmonary
22
Q
Treatment of Goodpasture syndrome is
A
1-plasmapheresis and glucocorticoids.
2-Cyclophosphamide may also help.
23
Q
IgA nephropathy and Henoch-Schönlein purpura (HSP) have a common pathophysiology and renal presentation, but differ in that HSP also shows signs of
A
systemic vasculitis
24
Q
IgA nephropathy (IgAN) is most commonly seen in Asian or native Americans age <35. It has 2 possible presentations:
A
- Mild or gross hematuria appearing 1–2 days after a upper respiratory infection resolves spontaneously in 30% of patients.
- Hematuria and non-nephrotic proteinuria without infectious precedent which gradually progresses to end-stage renal disease (ESRD) (more insidious form)
25
IgA nephropathy and Henoch-Schönlein purpura Compared to poststreptococcal GN, where renal involvement occurs
1–2 weeks later or longer after a sore throat
26
Hypertension is common, as in most GN. About 40 50% of IgAN patients progress to ............
Renal biopsy shows proliferation with .......................
ESRD
| IgA deposits.
27
HSP has a similar presentation and biopsy, but also shows a
skin rash or other vasculitic symptoms
28
Keys to diagnosis include
1-hematuria, 1–2 day association with URI (for IgAN),
| 2-and vasculitic rash, hematuria (for HSP).
29
Management of Henoch-Schönlein purpura
Renal biopsy is required if renal failure or proteinuria present. In HSP, skin biopsy is best
30
Treatment of Henoch-Schönlein purpura
no proven treatment. In the presence of proteinuria, give ACE inhibitors/ARB. If nephrotic, try glucocorticoids.
31
define Lupus nephritis
is a constellation of glomerular diseases associated with SLE.
32
clinical presentation of Lupus nephritis
There may be asymptomatic proteinuria or hematuria, nephrotic syndrome (secondary membranous GN) or
there may be severe nephritic syndrome with progressive renal failure eventually requiring dialysis.
33
the key to planning therapy and prognosis is
Biopsy
34
Key to diagnosis of Lupus nephritis:
nephritic or nephrotic syndrome with SLE diagnostic criteria;
35
The most accurate test is
biopsy
36
Treatment:
Glucocorticoids with mycophenolate for severe proliferative disease (nephritic).
Mycophenolate is superior to cyclophosphamide and has fewer side effects.
37
best test of Lupus nephritis is
double-stranded DNA levels and low complement levels during disease flares.
38
Idiopathic rapidly progressive glomerulonephritis (RPGN) presents with
```
nephritic syndrome (occasionally nephrotic as well) and relentless subacute renal failure, with the serum
creatinine rising over 1–2 weeks.
```
39
An early ............ ............... is critical to diagnosis, and shows ............... ............... ...............(“............... ...............”).
renal biopsy
| epithelial cell crescents (“crescentic GN”).
40
The key to diagnosis is rising rising ................. ; the best test is ................... ................. .
Treatment is ................... (start early to protect GFR) and .......................... (start after biopsy).
creatinine
renal biopsy
glucocorticoid
cyclophosphamide
41
Nephrotic diseases are characterized by
heavy proteinuria and may be primary or secondary to
| other systemic disease
42
Nephrotic diseases are often accompanied by a cluster of
metabolic abnormalities (termed the nephrotic syndrome).
43
The nephrotic syndrome is defined as
the presence of GN sufficient to produce
1-a level of proteinuria >3.5 grams per 24 hrs,
2-hyperlipidemia,
3-edema,
4-and low serum albumin.
44
Over 50% of nephrotic syndrome is associated with
a systemic disease, esp. DM.
45
Proteinuria arises because the damaged glomerular basement membrane loses
its negative charges; negatively charged albumin and key serum proteins then spill into the urine.
46
Proteinuria may lead to
1-hypoalbuminemia
| 2- and low serum oncotic pressure.
47
Complications of the nephrotic syndrome include:
1-Edema.
2-Hyperlipidemia and increased atherosclerosis.
3-Hypercoagulable states or thrombophilia
4-Spontaneous arterial or venous thrombosis due to hypercoagulability.
•5-Iron, copper, and zinc deficiency
48
Edema in nephrotic syndrome is due to
increased salt and water retention by the kidney, as well as low oncotic pressure in the serum.
49
Hyperlipidemia and increased atherosclerosis, in nephrotic syndrome is due to
most likely from the urinary loss of the lipoprotein markers or signals on the surface of chylomicrons and LDL that lead to the clearance of these lipids from the bloodstream.
50
Hypercoagulable states or thrombophilia, in nephrotic syndrome is due to
the urinary loss of natural anticoagulant
| proteins such as antithrombin, protein C, and protein S.
51
Spontaneous arterial or venous thrombosis in nephrotic syndrome is due to
hypercoagulability
52
Iron, copper, and zinc deficiency in nephrotic syndrome is due to
as a result of the urinary loss of their transport proteins such as transferrin and ceruloplasmin.
53
Diagnosis of nephrotic syndrome is based on the presence of
1->3.5 gm per 24 hrs protein in the urine (measured on 24-hour urine collection or a spot urine protein/creatinine ratio),
2-low serum albumin,
3-edema,
4-hyperlipidemia
54
The urinalysis will commonly only show
4+ protein, although some mild hematuria may be seen in several of the nephrotic glomerular
diseases.
55
The key to specific diagnosis is ............ ............. This may be deferred in diabetic nephropathy with a ................ ....................
renal biopsy
| typical history
56
Treatment
1-Control of the underlying disease, usually with glucocorticoids in the primary disorders.
2-If steroids do not work, add cyclophosphamide or mycophenolate. Azathioprine may be useful.
3-An ACE inhibitor or angiotensin receptor blocker (ARB) is used for all patients with proteinuria, but they do not reverse the underlying disease.
57
The following may also be helpful:
• Diuretics for .................
• ACE inhibitors/ARBs (equal efficacy) for control of .................... and ..........................
• Statins for ....................
• Anticoagulation if DVT or PE happen
• Good protein-calorie nutrition. Protein restriction is NOT indicated.
edema
control of proteinuria and hypertension
hyperlipidemia
58
Focal-Segmental Glomerulosclerosis (FSGS). The most common cause of ................ ................ in adults in the USA.
Secondary forms are seen with HIV (HIV nephropathy), the use of .................. as well as ............ ................(possibly due to hyperfiltration)
nephrotic syndrome
| heroin as well as morbid obesity
59
Treatment of Focal-Segmental Glomerulosclerosis
| is
glucocorticoids (20–40% response); may progress to ESRD over 5–10 years
60
Membranous Glomerulopathy. Most are .................... ..................
Secondary forms associated with SLE,
1-idiopathic (primary).
2-SLE
3-cancers such as lymphoma or breast cancer, infections such as endocarditis or chronic hepatitis B or C,
4-and drugs such as NSAIDs, penicillamine, gold salts, and NSAIDs.
61
Treatment of Membranous Glomerulopathy
glucocorticoids (30-50% response)
62
Minimal Change Disease. The most common nephrotic disease
in children (90-95%); may account for 15% of adult disease.
63
Usually primary, but NSAIDs and Hodgkin lymphoma have been associated with secondary disease. Light microscopy is normal and electron microscopy
is needed to see
fusion of foot processes.
64
Treatment:
High glucocorticoid response, esp. in children. The disease is often treated in kids without biopsy, with biopsy reserved for non-responders.
65
Adults are biopsied because of
wider differential diagnosis.
66
Membranoproliferative GN (also see cryoglobulinemia in Nephritic Diseases). Now largely type 1, associated with c
hronic hepatitis C and B; with or without cryoglobulinemia and vasculitis.
67
Renal presentation is nephritic and/or nephrotic Shows
low serum complement levels.
68
Routine urine dipstick detects only albumin and not .......... ............... or .........-............. ............(which must be done with urine immune electrophoresis).
Lipiduria may lead to an appearance of “Maltese crosses” in the urine.
light chains or Bence-Jones protein
69
Diabetic nephropathy is by far most common glomerular disease in developed countries.
The incidence of nephropathy is directly proportional to the
duration of the diabetes, and it normally appears as microalbuminuria after at least 10 years of type 1 or type 2 DM.
70
Microalbuminuria (50–300 mg/24 hours) is detected using
the spot urine albumin/creatine ratio, NOT the routine urinalysis, which is insensitive to low degrees of proteinuria
71
Screen all diabetic patients annually for microalbuminuria. Following the appearance of microalbumin, the proteinuria worsens, eventually becomes ................................ , followed by
worsening renal function with rising............................... Over 5-10 years the patient progresses to dialysis-requirement or transplantation. The leading cause of death is cardiac disease due to accelerated ........................... Other complications include ...................and ...........................................................
```
nephrotic (>3.5 grams)
serum creatinine
atherosclerosis
hyperkalemia
type IV renal tubular acidosis
```
72
Keys to diagnosis include
1-DM for at least 10 years; microalbuminuria
2-or (later) nephrotic syndrome
3-or decreased GFR.
73
Although a renal biopsy is the most accurate test for ............... involvement in diabetes, it is not routinely performed unless there is the possibility of another
disease causing the renal failure.
renal
74
Treatment of Diabetic nephropathy includes
tight control of diabetes and BP (<130/80 mm Hg); ACE inhibitor/ARB and statins for hyperlipidemia.
75
Renal amyloidosis occurs when amyloid proteins deposit in the glomerulus, causing damage to
the GBM, leading to decline in GFR, albuminuria, and the nephrotic syndrome.
76
There are 2 types of amyloidosis:
1-Amyloid light-chain (AL): plasma cell dyscrasia causing deposition of protein derived from immunoglobulin light chains; may be associated with multiple myeloma
2-Amyloid A (AA): amyloid is produced in association with a chronic infection, or rheumatoid diseases such as rheumatoid arthritis or IBD
77
Most patients will also have extrarenal manifestations like:
1-GI tract: diarrhea, malabsorption
2-Heart: restrictive cardiomyopathy, rhythm disorders, and heart block
3-ENT: large tongue (macroglossia)
4-Neuro: carpal tunnel syndrome, peripheral neuropathy
5-Muscles: weakness
78
The key to diagnosis is biopsy of an involved organ such as the
1-fat pad,
2-rectum,
3-nerves,
4-or kidney.
79
Congo red testing shows green birefringence. Patients with AL amyloid will also have
1-elevated urine
2-and serum light chains typical of myeloma,
3-and possible hypercalcemia.
80
Treatment is for the underlying malignancy or inflammation/infection. This is often very difficult. With AL amyloid, .................... and ................ can control protein production.
melphalan and prednisone
81
define Hypertensive nephrosclerosis
is the progressive chronic kidney disease associated with long-standing, poorly controlled hypertension.
82
While previously a common cause of ESRD in
| the United States, it is now less so, due to more extensive treatment of
hypertension
83
Patients’ CKD is often attributed to “hypertension” when in fact the ....................... is secondary to a
(potentially treatable) glomerular disease.
hypertension
84
If the hypertension is untreated, proteinuria and renal insufficiency progress gradually (over decades) to
dialysis requirement.
85
ACE inhibitors are the preferred antihypertensive due to their ............... ...................... effect in CKD.
renal protective
86
define Alport syndrome
is a glomerular disease due to genetic defect in type IV collagen, which structurally underlies the glomerular basement membrane
87
Patients present with the combination of mild
hematuria and proteinuria, along with ear (sensorineural hearing loss) and eye abnormalities.
88
Men are more susceptible to disease, as they .......................................... It may progress to dialysis-requirement. There is no treatment.
single mutated X chromosome
89
Hemolytic-uremic syndrome/idiopathic thrombocytopenic purpura (HUS/TTP) are
thrombotic microangiopathies that may present with small platelet clots in the renal microvessels,
90
causing secondary glomerular inflammation and renal failure. There is typically
1-acute renal failure,
2-mild hematuria,
3-and low-grade proteinuria (non-nephrotic).
91
Treatment OF Hemolytic-uremic syndrome is
for the underlying disorder
