Lecture 18 Glomerular Disease- Clinical Flashcards
Define glomerulonephritis
Immune-mediated disorders that affect the glomeruli
Name the main features of Glomerulonephritis
- Haematuria (non-visible or visible)
- Proteinuria (low grade or nephrotic)
- Hypertension
- Renal impairment
Define a Nephritic State
• Loss of blood
Active urine sediment: haematuria, dysmorphic RBCs, cellular casts
• Hypertension- salt and water retention and vasoactive hormone release
• Renal impairment
• Inflammation disrupting GBM= haematuria (cola-coloured urine)
• Reduced GFR
Define a Nephrotic State
Loss of protein
• Oedema
• Increase in hydrostatic pressure in the capillaries
• Nephrotic range proteinuria: >3.5g/day or 350mg/mmol creatinine
• Hypoalbuminemia: serum albumin <35g/L-
• Dyslipidemia
Define the term diffuse in terms of pathology
> 50% of glomeruli affected
Define Focal in term of pathology
<50% of glomeruli affected
Define global in terms of pathology
All glomerulus affected
Define segmental in term of pathology
Part of the glomerulus affected
Name non-proliferative glomerulonephritis
Minimal change disease
Membranous nephropathy
FSGS
Name proliferative glomerulonephritis
Mesangioproliferative GN
Membranoproliferative GN
Diffuse proliferative GN
Crescentic GN
Are Nephritic syndrome proliferative or non proliferative
Proliferative
is IgA nephropathy Nephrotic or nephritic
Nephritic
How does IgA Nephropathy cause haematuria
- IgA is stuck within the mesanagium and does not get filtered t the urine and it becomes clogged
- IgA – ‘irritates’ mesangial cells and causes them to proliferate and produce more matrix
What is post infectious glomerulonephritis
- Follows 10-21 days after infection typically of throat or skin.
- Most commonly with Lancefield group A Streptococci.
How is post infectious glomerulonephritis treated
– Antibiotics for infection, debatable.
– Loop diuretics such as frusemide for oedema (if there is oedema).
– Anti-hypertensives e.g. vasodilator drugs.
Name 3 causes of crescentic glomerulonephritis
ANCA-associated Anti-GBM IgA vasculitis POst infection glomerulonephritis SLE
What are the 3 main types of ANCA-asscoaued glomerulonephritis
- Microscopic polyangiitis.
- Granulomatosis with polyangiitis.
- Eosinophilic granulomatosis with polyangiitis
What is Anti-GBM disease
rare crescentic glomerulonephritis
What is Good Pasture’s syndrome
Nephritis + lung haemorrhage
How is anti-GBM treated
aggressive immunesuppression: steroid, plasma exchange, and cyclophosphamide.
Name 3 causes of Nephrotic syndrome (non-proliferative)
- Minimal change disease
- Focal and segmental glomerulonephritis
- Membranous Nephropathy
What is the general management of Nephrotic syndrome
- Treat oedema: salt and fluid restriction and loop diuretics.
- Hypertension: use Renin-Angiotensin-Aldosterone-blockade.
- Reduce risk of thrombosis: Heparin or Warfarin.
- Reduce risk of infection e.g. pneumococcal vaccine.
- Treat dyslipidemia e.g. statins.
Minimal change disease is commonly seen in what age group
Children
What are the clinical features of minimal change disease
- Sudden onset of oedema – days.
- Complete loss of proteinuria with steroids.
- Relapse occurs in two thirds of patients.
How is minimal change disease treated
- Prednisolone – 1mg/kg for up to 16 weeks.
* Once remission achieved , slow taper over 6 months.
If there is an initial relapse in minimal change disease how is that treated
• Initial relapse treated with further steroid course.
If there are subsequent relapses in minimal change disease how is that treated
- Cyclophosphamide
- Cyclosporin- immunosuppression (calcineurin inhibitor)
- Tacrolimus- immunosuppression (calcineurin inhibitor)
- Mycophenolate mofetil (Anti-proliferative)
- Rituximab (depleting agent)
What is the prognosis for minimal change disease
- Despite relapsing behaviour, prognosis is favourable.
- Risk of end stage kidney disease is low.
- Steroids toxicity as multiple exposure.
What is focal and segmental Glomerulosclerosis
- Presents with nephrotic syndrome.
* Pathology reveals focal and segmental sclerosis with distinctive patterns
What are the distinctive patterns of focal and segmental Glomerulosclerosis
• tip lesion, collapsing, cellular, perihilar
What is the prognosis of focal and segmental Glomerulosclerosis
- Generally steroid resistant.
* High chance of progression to end stage kidney disease.
How is focal and segmental Glomerulosclerosis treated
- Treat oedema: salt and fluid restriction and loop diuretics.
- Hypertension: use Renin-Angiotensin-Aldosterone-blockade.
- Reduce risk of thrombosis: Heparin or Warfarin.
- Reduce risk of infection e.g. pneumococcal vaccine.
- Treat dyslipidemia e.g. statins.
- Trail of steroids, positive response , even partial remission, carries better prognosis.
- Alternative options: cyclosporin, cyclophosphamide, and Rituximab
What is membranous nephropathy
- Commonest cause of nephrotic syndrome in adults.
- 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 (C3), which punches holes in filter
- Leaky filter now allows albumin to be filtered into urine nephrotic syndrome
What is the treatment for membranous nephropathy
- Treat oedema: salt and fluid restriction and loop diuretics.
- Hypertension: use Renin-Angiotensin-Aldosterone-blockade.
- Reduce risk of thrombosis: Heparin or Warfarin.
- Reduce risk of infection e.g. pneumococcal vaccine.
- Treat dyslipidemia e.g. statins.
- Immunesuppression if symptomatic nephrotic syndrome, rising proteinuria or deteriorating renal function.
- Cyclophosphamide and steroids (alternate months) for 6 months.
- Tacrolimus.
- Rituximab.
