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Nephrology > Nephritic glomerular diseases > Flashcards

Nephritic glomerular diseases Flashcards

1
Q

in Nephritic glomerular disease is limited to

A

the glomerulus

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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

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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

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4
Q

Poststreptococcal GN occurs in 10–15% of patients with

A

pharyngitis

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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

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6
Q

The key to diagnosis is an association with

A

infection

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7
Q

The best initial test is the

A

antistreptolysin (ASO) or antihyaluronic acid (AHT)

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8
Q

Complement levels, particularly C3, are

A

low.

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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

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10
Q

Treatment of Poststreptococcal GN is

A

supportive (management of fluid overload and hypertension with diuretics). Most cases resolve spontaneously.

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11
Q

…………. will eradicate the organism from the pharynx

A

Antibiotics

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12
Q

……………… are sometimes used for unusual persistence of proteinuria or renal failure in adults.

A

Glucocorticoids

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13
Q

define Goodpasture syndrome (GPS)

A

is an idiopathic renal and lung disease characterized by a unique anti-glomerular basement membrane antibody

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14
Q

clinical Presentation includes

A

hematuria and hemoptysis.

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15
Q

Aside from the………….. and …………….. , GPS does not affect other sites in the body

A

lungs and kidneys

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16
Q

thus an absence of skin or eye findings is a clue to

A

the diagnosis.

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17
Q

When there is lung involvement (65%), patients present with

A

1-hemoptysis,
2-cough,
3-shortness of breath.

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18
Q

The key to diagnosis of Goodpasture syndrome (GPS) is

A

nephritic-pulmonary syndrome

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19
Q

The best initial test for nephritic-pulmonary syndrome is

A

the level of antibasement membrane antibodies to type IV collagen.

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20
Q

The single most accurate test is

A

lung or kidney biopsy, which will show linear deposits on immunofluorescence

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21
Q

Do lung biopsy, not renal, if there is …………………… involvement.

A

pulmonary

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22
Q

Treatment of Goodpasture syndrome is

A

1-plasmapheresis and glucocorticoids.

2-Cyclophosphamide may also help.

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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

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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)
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25
Q

IgA nephropathy and Henoch-Schönlein purpura Compared to poststreptococcal GN, where renal involvement occurs

A

1–2 weeks later or longer after a sore throat

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26
Q

Hypertension is common, as in most GN. About 40 50% of IgAN patients progress to …………
Renal biopsy shows proliferation with …………………..

A

ESRD

IgA deposits.

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27
Q

HSP has a similar presentation and biopsy, but also shows a

A

skin rash or other vasculitic symptoms

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28
Q

Keys to diagnosis include

A

1-hematuria, 1–2 day association with URI (for IgAN),

2-and vasculitic rash, hematuria (for HSP).

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29
Q

Management of Henoch-Schönlein purpura

A

Renal biopsy is required if renal failure or proteinuria present. In HSP, skin biopsy is best

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30
Q

Treatment of Henoch-Schönlein purpura

A

no proven treatment. In the presence of proteinuria, give ACE inhibitors/ARB. If nephrotic, try glucocorticoids.

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31
Q

define Lupus nephritis

A

is a constellation of glomerular diseases associated with SLE.

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32
Q

clinical presentation of Lupus nephritis

A

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.

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33
Q

the key to planning therapy and prognosis is

A

Biopsy

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34
Q

Key to diagnosis of Lupus nephritis:

A

nephritic or nephrotic syndrome with SLE diagnostic criteria;

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35
Q

The most accurate test is

A

biopsy

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36
Q

Treatment:

A

Glucocorticoids with mycophenolate for severe proliferative disease (nephritic).
Mycophenolate is superior to cyclophosphamide and has fewer side effects.

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37
Q

best test of Lupus nephritis is

A

double-stranded DNA levels and low complement levels during disease flares.

38
Q

Idiopathic rapidly progressive glomerulonephritis (RPGN) presents with

A 
nephritic syndrome (occasionally nephrotic as well) and relentless subacute renal failure, with the serum
creatinine rising over 1–2 weeks.
39
Q

An early ………… …………… is critical to diagnosis, and shows …………… …………… ……………(“…………… ……………”).

A

renal biopsy

epithelial cell crescents (“crescentic GN”).

40
Q

The key to diagnosis is rising rising …………….. ; the best test is ………………. …………….. .
Treatment is ………………. (start early to protect GFR) and …………………….. (start after biopsy).

A

creatinine
renal biopsy
glucocorticoid
cyclophosphamide

41
Q

Nephrotic diseases are characterized by

A

heavy proteinuria and may be primary or secondary to

other systemic disease

42
Q

Nephrotic diseases are often accompanied by a cluster of

A

metabolic abnormalities (termed the nephrotic syndrome).

43
Q

The nephrotic syndrome is defined as

A

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
Q

Over 50% of nephrotic syndrome is associated with

A

a systemic disease, esp. DM.

45
Q

Proteinuria arises because the damaged glomerular basement membrane loses

A

its negative charges; negatively charged albumin and key serum proteins then spill into the urine.

46
Q

Proteinuria may lead to

A

1-hypoalbuminemia

2- and low serum oncotic pressure.

47
Q

Complications of the nephrotic syndrome include:

A

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
Q

Edema in nephrotic syndrome is due to

A

increased salt and water retention by the kidney, as well as low oncotic pressure in the serum.

49
Q

Hyperlipidemia and increased atherosclerosis, in nephrotic syndrome is due to

A

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
Q

Hypercoagulable states or thrombophilia, in nephrotic syndrome is due to

A

the urinary loss of natural anticoagulant

proteins such as antithrombin, protein C, and protein S.

51
Q

Spontaneous arterial or venous thrombosis in nephrotic syndrome is due to

A

hypercoagulability

52
Q

Iron, copper, and zinc deficiency in nephrotic syndrome is due to

A

as a result of the urinary loss of their transport proteins such as transferrin and ceruloplasmin.

53
Q

Diagnosis of nephrotic syndrome is based on the presence of

A

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
Q

The urinalysis will commonly only show

A

4+ protein, although some mild hematuria may be seen in several of the nephrotic glomerular
diseases.

55
Q

The key to specific diagnosis is ………… …………. This may be deferred in diabetic nephropathy with a ……………. ………………..

A

renal biopsy

typical history

56
Q

Treatment

A

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
Q

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.

A

edema
control of proteinuria and hypertension
hyperlipidemia

58
Q

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)

A

nephrotic syndrome

heroin as well as morbid obesity

59
Q

Treatment of Focal-Segmental Glomerulosclerosis

is

A

glucocorticoids (20–40% response); may progress to ESRD over 5–10 years

60
Q

Membranous Glomerulopathy. Most are ……………….. ………………
Secondary forms associated with SLE,

A

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
Q

Treatment of Membranous Glomerulopathy

A

glucocorticoids (30-50% response)

62
Q

Minimal Change Disease. The most common nephrotic disease

A

in children (90-95%); may account for 15% of adult disease.

63
Q

Usually primary, but NSAIDs and Hodgkin lymphoma have been associated with secondary disease. Light microscopy is normal and electron microscopy
is needed to see

A

fusion of foot processes.

64
Q

Treatment:

A

High glucocorticoid response, esp. in children. The disease is often treated in kids without biopsy, with biopsy reserved for non-responders.

65
Q

Adults are biopsied because of

A

wider differential diagnosis.

66
Q

Membranoproliferative GN (also see cryoglobulinemia in Nephritic Diseases). Now largely type 1, associated with c

A

hronic hepatitis C and B; with or without cryoglobulinemia and vasculitis.

67
Q

Renal presentation is nephritic and/or nephrotic Shows

A

low serum complement levels.

68
Q

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.

A

light chains or Bence-Jones protein

69
Q

Diabetic nephropathy is by far most common glomerular disease in developed countries.
The incidence of nephropathy is directly proportional to the

A

duration of the diabetes, and it normally appears as microalbuminuria after at least 10 years of type 1 or type 2 DM.

70
Q

Microalbuminuria (50–300 mg/24 hours) is detected using

A

the spot urine albumin/creatine ratio, NOT the routine urinalysis, which is insensitive to low degrees of proteinuria

71
Q

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 …………………………………………………..

A 
nephrotic (>3.5 grams)
 serum creatinine
atherosclerosis
hyperkalemia 
type IV renal tubular acidosis
72
Q

Keys to diagnosis include

A

1-DM for at least 10 years; microalbuminuria
2-or (later) nephrotic syndrome
3-or decreased GFR.

73
Q

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.

A

renal

74
Q

Treatment of Diabetic nephropathy includes

A

tight control of diabetes and BP (<130/80 mm Hg); ACE inhibitor/ARB and statins for hyperlipidemia.

75
Q

Renal amyloidosis occurs when amyloid proteins deposit in the glomerulus, causing damage to

A

the GBM, leading to decline in GFR, albuminuria, and the nephrotic syndrome.

76
Q

There are 2 types of amyloidosis:

A

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
Q

Most patients will also have extrarenal manifestations like:

A

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
Q

The key to diagnosis is biopsy of an involved organ such as the

A

1-fat pad,
2-rectum,
3-nerves,
4-or kidney.

79
Q

Congo red testing shows green birefringence. Patients with AL amyloid will also have

A

1-elevated urine
2-and serum light chains typical of myeloma,
3-and possible hypercalcemia.

80
Q

Treatment is for the underlying malignancy or inflammation/infection. This is often very difficult. With AL amyloid, ……………….. and ……………. can control protein production.

A

melphalan and prednisone

81
Q

define Hypertensive nephrosclerosis

A

is the progressive chronic kidney disease associated with long-standing, poorly controlled hypertension.

82
Q

While previously a common cause of ESRD in

the United States, it is now less so, due to more extensive treatment of

A

hypertension

83
Q

Patients’ CKD is often attributed to “hypertension” when in fact the ………………….. is secondary to a
(potentially treatable) glomerular disease.

A

hypertension

84
Q

If the hypertension is untreated, proteinuria and renal insufficiency progress gradually (over decades) to

A

dialysis requirement.

85
Q

ACE inhibitors are the preferred antihypertensive due to their …………… …………………. effect in CKD.

A

renal protective

86
Q

define Alport syndrome

A

is a glomerular disease due to genetic defect in type IV collagen, which structurally underlies the glomerular basement membrane

87
Q

Patients present with the combination of mild

A

hematuria and proteinuria, along with ear (sensorineural hearing loss) and eye abnormalities.

88
Q

Men are more susceptible to disease, as they …………………………………… It may progress to dialysis-requirement. There is no treatment.

A

single mutated X chromosome

89
Q

Hemolytic-uremic syndrome/idiopathic thrombocytopenic purpura (HUS/TTP) are

A

thrombotic microangiopathies that may present with small platelet clots in the renal microvessels,

90
Q

causing secondary glomerular inflammation and renal failure. There is typically

A

1-acute renal failure,
2-mild hematuria,
3-and low-grade proteinuria (non-nephrotic).

91
Q

Treatment OF Hemolytic-uremic syndrome is

A

for the underlying disorder

Nephrology (5 decks)

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