1. Glomerular diseases Flashcards

1
Q

What are the clinical manifestations of glomerular diseases?

A
  • Hematuria and/or proteinuria,
  • deterioration of kidney function,
  • development of hypertension,
  • edema (peripheral, periorbital),
  • hypercoagulability,
  • appearance of systemic symptoms.
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2
Q

What are the clinical features of acute nephritis syndrome?

A
  • Decrease in eGFR in a few days
  • oliguria
  • microscopic or macroscopic hematuria, dysmorphic RBC
  • subnephrotic proteinuria
  • normal or slightly low serum albumin,
  • generalized edema,
  • high blood pressure
  • may include headache and anorexia.
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3
Q

What are the clinical features of rapid progressive glomerulonephritis?

A
  • Decrease in eGFR in a few days to 1-2 months,
  • can have oliguria,
  • mostly microscopic hematuria with dysmorphic RBCs and acanthrocytes,
  • subnephrotic proteinuria,
  • normal or slightly low serum albumin,
  • maybe mild edema,
  • normal blood pressure,
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4
Q

What are the systemic symptoms associated with Rapid Progressive Glomerulonephritis (RPGN) syndrome?

A

Fever, weight loss, joint pain, muscle pain, weakness, palpable purpura.

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5
Q

What are the only presenting symptoms in patients with Chronic (asymptomatic) nephritis?

A
  • Microscopic hematuria with dysmorphic RBC
  • high BP
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6
Q

What is the general treatment for nephritis syndromes?

A

Fluid status, table salt and fluid intake reduction, Furosemide, regulation of blood pressure.

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7
Q

What is the effect of ACEi/ARB on eGFR?

A

Decrease.

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8
Q

What is the typical fluid intake reduction for patients with nephritis syndromes?

A

500-1000 ml/day.

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9
Q

What is the typical table salt intake for patients with nephritis syndromes?

A

less than 5 gr/day.

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10
Q

What is the definition of Nephrotic syndrome?

A

Nephrotic syndrome is characterized by heavy proteinuria (> 3 g/day), hypoalbuminemia, and pronounced edema (face and limbs)

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11
Q

What is the typical urine volume in Nephrotic syndrome?

A

Urine volume is normal in Nephrotic syndrome.

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12
Q

What is the level of Hematuria in Nephrotic syndrome?

A

Hematuria is not typical in Nephrotic syndrome.

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13
Q

What is the level of Serum albumin in Nephrotic syndrome?

A

Serum albumin is low in Nephrotic syndrome.

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14
Q

What is the level of Blood pressure in Nephrotic syndrome?

A

Blood pressure is normal or low in Nephrotic syndrome.

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15
Q

What are the consequences of proteinuria in Nephrotic syndrome?

A

The consequences of proteinuria in Nephrotic syndrome include generalized edema and hyperlipidemia.

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16
Q

What is the general treatment for Nephrotic syndrome?

A
  • weight loss,
  • liquid and salt restriction,
  • diuretics,
  • proteinuria reducing medications such as ACE inhibitors or ARBs.
  • anticoagulant treatment
  • lipid lowering drugs
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17
Q

What is the recommended protein restriction for Nephrotic syndrome patients?

A

The recommended protein restriction for Nephrotic syndrome patients is 0.8 g/kg/day.

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18
Q

What is the recommended anticoagulant treatment for Nephrotic syndrome patients with low serum albumin?

A

The recommended anticoagulant treatment for Nephrotic syndrome patients with low serum albumin is LMWH.

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19
Q

What are the recommended lipid-lowering drugs for patients with normal kidney function?

A

Fibrates - otherwise use statins

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20
Q

What are the recommended serum tests for investigating glomerulopathies?

A

Creatinine, Urea, Ions, Albumin, CRP, AST.

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21
Q

What are the recommended immunoglobulin tests for investigating glomerulopathies?

A

IgA, IgG, IgM, cryoglobulin.

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22
Q

What are the recommended complement tests for investigating glomerulopathies?

A

C3, C4.

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23
Q

What are the recommended serum light chain tests for investigating glomerulopathies?

A

ELFO, lamda, kappa

24
Q

What are the recommended antibody tests for investigating glomerulopathies?

A

ANA, anti-dsDNA, ANCA, antiPLA2R (antibodies to phospholipase A2)

25
Q

What are the recommended protein excretion tests for investigating glomerulopathies?

A

mg/24 h or ~mg/mmol (albumin/creatinine or total protein/creatinine).

26
Q

What are the recommended urine tests for investigating glomerulopathies?

A

Urine sediment test, Stix, protein ELFO.

27
Q

What are the two histological classifications of glomerular diseases?

A

Proliferative and non-proliferative.

28
Q

What is the clinical picture associated with poststreptococcus GN?

A
  • Acute nephrITIC syndrome
  • Proliferative
  • Can be isolated hematuria (asymptomatic).
29
Q

What is the histology associated with poststreptococcus GN?

A
  • Endocapillary proliferative glomerulonephritis
  • Granular deposits
30
Q

What is ANCA vasculitis?

A
  • Rapidly progressing glomerulonephritis
  • Proliferative
  • Autoantibodies against MPO or PR3 (neutrophil proteins)
31
Q

Histological findings in ANCA vasculitis?

A
  • Extracapillary proliferation
  • Crescent formation
  • Pauci immune (!)
32
Q

Clinical picture of Goodpasture syndrome

A
  • RPGN (nephritic syndrome)
  • Proliferation
33
Q

Histological findings of goodpasture syndrome

A
  • Extracapillary proliferation
  • LINEAR depositions
34
Q

What are the special laboratory findings in a patient with SLE nephritis?

A
  • ANA-Screen positive,
  • Anti-dsDNA and anti-C1q elevated,
  • C4 and C3 decreased.
35
Q

What is the histological finding in SLE nephritis?

A
  • Mesangial and endocapillary proliferation w/ or w/o crescents
  • Granular deposition
36
Q

What is the histological finding in IgA nephropathy?

A
  • Mesangial proliferation
  • Granular deposition
37
Q

What is the clinical presentation of Sch̦nlein-Enoch Purpura?

A
  • Mostly nephritic syndrome, but it can also present as mixed nephrotic / nephritic.
  • Palpable purpura, joint pain, abdominal pain, bleeding with lower GI, hematuria, and deteriorating eGFR.
38
Q

What is the clinical presentation of membranoproliferative/mesangiocapillaris GN?

A
  • Almost anything but most often mixed or nephritic syndrome.
  • Both proliferative and non proliferative abnormalities
39
Q

What is the clinical picture of minimal change disease?

A
  • Nephrotic syndrome.
  • Non proliferative
40
Q

What is the clinical picture of focal segmental glomerulosclerosis?

A
  • Nephrotic syndrome with or without decreasing GFR.
  • Non proliferative
41
Q

What disease can cause minimal change disease?

A

Hodgkin’s disease.

42
Q

What are other causes of focal segmental glomerulosclerosis?

A

HIV, heroin abuse, extremely low birth weight, extreme obesity, bodybuilding, obstructive uropathy.

43
Q

What are the histological findings in membranous nephropathy?

A

Granular deposition, thickening of basement membrane

44
Q

What is the clinical picture of membranous nephropathy?

A
  • Nephrotic syndrome.
  • Non proliferative
45
Q

What are secondary causes of membranous nephropathy?

A

Solid tumor/chronic infection/autoimmune disease.

46
Q

What is the clinical picture of diabetic nephropathy?

A
  • Nephrotic syndrome/isolated proteinuria with or without decreasing GFR.
  • Non proliferative
47
Q

What is the clinical picture of a patient with amyloidosis?

A
  • Nephrotic syndrome (with or without decreasing GFR)
  • Non proliferative
48
Q

What is the recommended treatment for acute nephritis

A
  • Supportive therapy
  • volume and salt absorption control,
  • loop diuretic (furosemide)
  • antihypertensive therapy (CCB; ACE inhibitors / ARB only with caution!),
  • renal replacement therapy.
49
Q

What is the recommended treatment for chronic nephritis?

A
  • Antihypertensive and anti-proteinuria therapy (ACE inhibitors / ARB),
  • fish oil
  • depending on the tissue p.o. steroids
50
Q

What is the recommended treatment for RPGN?

A
  • Aggressive immunosuppression is required
  • IV then per os steroids
  • rituximab maintenance therapy
51
Q

What is the recommended treatment for minimal change nephrosis?

A

Usually a good response to steroids.

52
Q

What is the recommended treatment for FSGS?

A

Longer-term steroid therapy is required.

53
Q

What is the recommended treatment for membranous nephropathy?

A

Steroid + Cyclophosphamide / Cyclosporine and in a therapy-resistant case use rituximab.

54
Q

What is the recommended treatment for diabetic nephropathy?

A

Adequate blood sugar control, ACE inhibitors, or ARB to control blood pressure and reduce proteinuria, and reduction of salt intake.

55
Q

What is the recommended treatment for amyloid (AL)?

A

Hematological treatment.

56
Q

What diseases typically manifest as RPGN?

A
  • SLE nephritis
  • ANCA vasculitis
  • Goodpasture syndrome