Glomerular disease (pathology) Flashcards

1
Q

What are the 3 layers of the glomerular membrane that form the filter barrier?

A

fenestrated endothelial cells membrane
basal lamina
podocytes

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2
Q

What are Mesangial cells?

A

Mesangial cells - ‘tree-like’ group of pericyte cells which support capillaries within the glomerulus

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3
Q

What is Glomerulonephritis?

A

Glomerulonephritis (GN) is a term used to refer to several kidney diseases (usually affecting both kidneys). Many of the diseases are characterised by inflammation either of the glomeruli or of the small blood vessels in the kidneys, hence the name, but not all diseases necessarily have an inflammatory component.

Primary (only affects glomerulus) or Secondary (other parts of body affected, e.g. SLE or Wegener’s)

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4
Q

What are 4 common presentations in glomerulonephritis?

A

Haematuria (blood in urine)

Heavy proteinuria (nephrotic syndrome) - acute

Slowly increasing proteinuria over many years - chronic

Acute renal failure – shown by rising creatinine

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5
Q

What are the 4 most common causes of haematuria in order of likelihood?

A
  • Urinary tract infection
  • Urinary tract stone
  • Urinary tract tumour
  • Glomerulonephritis
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6
Q

What type of glomerulonephritis is associated with IgA and complement component C3 deposits in the mesangial area of all glomeruli?

A

IgA glomerulonephritis

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7
Q

Describe the features of IgA glomerulonephritis?

A

IgA and component C3 deposits in mensangial area
IgA irritates these cells, causing them to proliferate and produce more matrix
This can compress the capillaries

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8
Q

Describe the prognosis for those with IgA glomerulonephritis

A

Usually self limiting

Can progress to chronic renal failure in small % of cases

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9
Q

To which type of glomerulonephritis is IgA glomerulonephritis one of?

A

Proliferative glomerulonephritis

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10
Q

What is Nephrotic syndrome?

A

Nephrotic syndrome is a collection of symptoms due to kidney damage. This includes protein in the urine, low blood albumin levels, high blood lipids, and significant swelling. Other symptoms may include weight gain, feeling tired, and foamy urine. Complications may include blood clots, infections, and high blood pressure

Causes include a number of kidney diseases such as focal segmental glomerulosclerosis, membranous nephropathy, and minimal change disease. It may also occur as a complication of diabetes or lupus. The underlying mechanism typically involves damage to the glomeruli of the kidney. Diagnosis is typically based on urine testing and sometimes a kidney biopsy. It differs from nephritic syndrome in that there are no red blood cells in the urine

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11
Q

List some potential causes of nephrotic syndrome

A

Causes include a number of kidney diseases such as focal segmental glomerulosclerosis, membranous nephropathy, and minimal change disease. It may also occur as a complication of diabetes or lupus. The underlying mechanism typically involves damage to the glomeruli of the kidney. Diagnosis is typically based on urine testing and sometimes a kidney biopsy. It differs from nephritic syndrome in that there are no red blood cells in the urine

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12
Q

Describe membranous glomerulonephritis

A
  • In membranous glomerulonephritis, IgG is stuck in membrane
  • IgG deposits itself between basal lamina and podocyte but cannot go further and is not filtered into urine
  • IgG is too big to be filtered into urine, but IgG activates complement protein C3 within the membrane, which punches holes in filter
  • Leaky filter now allows albumin to be filtered into urine à nephrotic syndrome
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13
Q

Describe the prognosis of membraneous glomerulonephritis

A

1/4 of patients have chronic renal failure within 10 years

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14
Q

What causes IgG production and accumulation in membranous glomerulonephrits?

A

o Unknown but can sometimes have underlying malignancy

o In many patients antigen is phospholipase A2 receptor – why this protein? – as yet unknown.

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15
Q

Describe the features of diabetic nephropathy

A
  • Diabetic nephropathy – Nodules of mesangial matrix known as Kimmelsteil-Wilson lesion found, which are made from gross excess of mesangial matrix forming nodules
  • Glycated molecules are deposited in the matrix within the basal lamina underlying the endothelium and in the mesangial matrix. These then become thickened and the basement membrane becomes leaky. The mesingeal matrix can also compress he capillaries. This condition is not caused by immune complexes.
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16
Q

Describe the prognosis for diabetic nephropathy

A

Diabetic nephropathy prognosis - inevitable decline to dialysis if:
o 1. Established diabetic nephropathy and if
o 2. Continued poor diabetic control

17
Q

List some potential causes of Crescentic glomerulonephritis

A

o Granulomatosis with polyangiitis (also known as Wegener’s granulomatosis)
o Microscopic polyarteritis (a disease very much like Wegeners)
o Antiglomerular basement membrane disease
o Many other forms of glomerulonephritis

18
Q

List a test used for Granulomatosis with polyangitis (formerly known as Wegeners granulomatosis)

A

serum test that shows presence of anti-neutrophil cytoplasmic antibodies (ANCA)

19
Q

What are anti-neutrophil cytoplasmic antibodies (ANCA)?

A
  • Not deposited in kidney
  • Antibodies directed against proteinase 3 and myeloperoxidase, 2 enzymes in primary granules of neutrophils
  • Antibodies produce tissue damage via interactions with primed neutrophils targeting endothelial cells.
  • Unknown why these antibodies form, could potentially be a form of autoimmunity
20
Q

Describe the prognosis for those with Granulomatosis with polyangitis (formerly known as Wegeners granulomatosis)

A
  • Fatal (mean survival 6 months) if left untreated

* Cyclophosphamide – 75% complete remission

21
Q

How is Granulomatosis with polyangitis (formerly known as Wegeners granulomatosis) treated?

A

Cyclophosphamide