Nepthritic syndrome Flashcards
Nephritis syndrome : Causes
- Children and Adolescents
- IgA nephropathy
- Post streptococcal glomerulonephrotos
- Haemolytic uraemia syndrome - Adults
- Systemic lupus erythematosis
- Goodpasture’s syndrome
- Rapidly progressive glomerulonephritis
Nephritic syndrome : definition
- Diseased caused by inflammation and damage to glomeruli of kidney
- Glomeruli become more permeable -> Allow RBCs into the urine -> Haematuria
3 . Clincial features :
* Haemturia
* Oliguria
* Oedema
* Hypertension
Nephritis syndrome : Clinical signs and symptom
- Haematuria / Proteinuria : Damaged, Permeable glomeruli
- Odaema, Hypertension : Decreased glomerular filtration rate
- Uraemia : less waste product excreted
Post streptococcal glomerularnephritis : definition
- Inflammation of glomeruli, complication of bacterial infection
- Arises several weeks after : Group A beta haemolytic streptococcus infection
Post streptococcal glomerularnephritis : Pathophysiology
- Type III hypersensitivity reaction
- IgG/IgM antibodies bind to bacterial antigens } form immune complex in the blood stream.
- Complex deposits in the glomerular basement membrane
- Immune complex deposits trigger immune reaction -> inflammatory cells /Complement system recruited -> Basement membrane damage
Post streptococcal glomerularnephritis : Cause
Group A beta haemolytic streptococcus infection
Post streptococcal glomerularnephritis : Risk factors
Children - 6 weeks following Impetigo, 1-2 weeks after a throat infection
Post streptococcal glomerularnephritis : Complication
- Post-streptococcal glomerulonephritis causes progress to acute, diffuse proliferative glomerulonephritis
- Renal failure
Post streptococcal glomerularnephritis : Investigation
1 .Bloods
Antibodies against group A strep : Anti streptolysin O antibodies
2 . Renal biopsy
* Light microscopy - mesangial proliferation
* Electron microscopy : Subepithelial deposits of immune complexes ‘Humps’
* Immunofluorescence : ‘Starry sky’ granular deposits of IgG complement in the basement membrane of the mesangium
Good pasture’s syndrome : Definition
Goodpasture syndrome is an autoimmune disease in which the immune system attacks the A3 chain of Type IV collagen present in the basement membrane
Good pasture’s syndrome : Pathophysiology
- Auto-antibody igG bind to A3 chain of type IV collagen and activate the complement system
- Inflammation and damage to the basement membrane of the kidney and the lungs.
Good pasture’s syndrome : Risk factor
- Environmental
Damage to collagen molecules - exposing antigenic regions of A3 chain - Infection, smoking, occupational risk - Genetic - HLA-DR15 strong genetic susceptibility
Good pasture’s syndrome : Incidence
20-30 years
Good pasture’s syndrome : Clinical features
Pulmonary manifestations occur before renal ones
1. Damaged alveolar basement membrane : Cough, haemoptysis and dyspnea
2. Damaged GBM of the kidneys : Nephritic syndrome
Good pasture’s syndrome : Investigations
Renal biopsy
1. Light microscopy : Crescentric glomerularnephritis
2. Electron microscopy : Diffuse thickening of the glomerular basement membrane
3. Immunofluorescence : Linear deposition along basement membrane
Good pasture’s syndrome : Management
- Immunosuppressant : Corticosteroid, Cyclophosphamide
- Plasmapheresis
Rapidly progressive glomerulonephritis : definition
Inflammation of the nephrons - crescent shaped proliferation of cells in the Bowman’s capsule
* This leads to renal failure within weeks - months
Rapidly progressive glomerulonephritis : Pathophysiology
- Inflammation damages the GBM
- Inflammatory mediators enter the Bowman’s space
- Trigger expansion of thin epithelial layer of cells into thick, crescent moon shape
Rapidly progressive glomerulonephritis : Causes
1 .Primary : Idiopathic
2 .Secondary
* Type 1 : anti GBM antibodies
-Goodpasture syndrome
-
Type II : Immune complexes deposition
-Poststreptococcal glomerulonephritis
-Lupus
-IgA nephropathy -
Type III : ANCA antibodies in the blood
-Vasculitis : Wegener’s granulomatosis etc
Rapidly progressive glomerulonephritis : Complication
If untreated - Rapid progression to acute renal failure
Rapidly progressive glomerulonephritis : Diagnosis
- Light microscopy : crescent shaped glomeruli dueto proliferation of epithelial cells in bowmans capsule
-
Immunofluorescence - differentiate types of RPG
* Type 1 : Linear pattern
Anti GBM Antibodies bind to collagen of the glomerular basement membrane
* Type II : Granular pattern
Result of immune complex deposition int he sub endothelium
* Type III : negative
No antibodies or immune complex deposition } only associated with ANCA in the blood
Rapidly progressive glomerulonephritis : Incidence
Affects adults in their 50s and their 60s
Typically has a poor prognosis if not treated early.
Which is the most common nephropathy worldwide?
IgA nephropathy / Berger disease
IgA nephropathy : Definition
Abnormal IgA forms and deposits in the kidneys resulting in kidney damage
