Flashcards in Glomerulonephritis Deck (49):
"Immune mediated disease of the kidneys affecting the glomeruli"
-Often with secondary interstitial damage"
Three main pathogenic mechanisms of GN
-Humoral - antibody mediated
-Cell mediated - (T cells)
-Inflammatory cells, mediators and complement
Damage to the selective membrane barrier causes what?
Haematuria &/or proteinuria
Damage to what cells causes proliferative lesions and red blood cells in urine
Damage to podocytes leads to what kind of lesion?
Protein in urine
How does the mesangium respond to injury?
Cause chemokine release and attract inflammatory cells
Damage to the podocytes causes what?
Atrophy and loss of size/charge of specific barrier
Damage to endothelial cells leads to what?
When testing urine proteins, how do you effectively quantify proteinuria?
Creatinine ratio with 24 hour urine
What other signs of GN can be seen through urine microscopy?
What measurement of proteinuria is definitive of nephrotic syndrome?
What reading would be considered asymptomatic proteinuria?
What non-renal condition might a patient present with suggestive of renal disease
Clinical symptoms/signs of Nephritic Syndrome
Acute renal failure
Oedema and fluid retention
Active urinary sediment
-(RBCs and Granular casts)
Indication of proliferative process affecting ENDOTHELIAL cells
Clinical Signs/symptoms of Nephrotic Syndrome
Usually normal renal function
Indication of NON proliferative process, affecting PODOCYTES
Complications of Nephrotic Syndrome
Infections - due to loss of opsonising antibodies
Renal Vein thrombosis
Vit d deficiency
Use of what long term drug can cause volume depletion complications in nephrotic syndrome?
Over-use of diuretics
Main cause of GN
Primary - Idiopathic
Causes of secondary GN
Examples of systemic diseases causing GN
ANCA associated systemic vasculitis, Good-pastures, HSP, lupus etc
Investigative histological tests required for classification of GN
What does the term proliferative vs non proliferative refer to?
Presence vs absence of proliferation of mesangial cells
Define focal vs diffuse in classification
Focal <50% of glomerulus affected
Diffuse >50% of glomerulus affected
What does it mean is biopsy is crescentic?
Presence of crescents noted on histology -
Epithelial cell extra-capillary proliferation
e.g. RPGN in vasculitis
What are the principles of treatment in GN?
- Reduce degree of proteinuria
- Induce remission of nephrotic syndrome
- Preserve long-term renal function
Non-immunosuppresive treatment for GN
Consider: anticoagulants, aspirin and anti-platelets
?Omega 3/fish oil
Target BP in GN with/without proteinuria
With - <120/75
Without - <130/80
Immunosuppresive Treatment for GN
General treatment of Nephrotic Patients
IV Albumin if volume depleted
Immunosuppression with aim to induce sustained remission
Main types of (primary) idiopathic GN
What is minimal change disease?
Most common cause of nephrotic syndrome in children
Normal renal biopsy with foot process fusion on EM
Does NOT cause progressive renal failure
May be caused by IL-13
94% achieve remission on oral steroid alone
What is Focal Segmental Glomerulosclerosis
Commonest cause of nephrotic syndrome in adults
Can be primary or secondary cause (HIV/heroin/obesity/)
Renal biopsy shows what name states on LM and complement deposition on IF
Remission in 60% with prolonged steroids
50% will progress to end stage renal failure in 10years
What is Membranous Nephropathy?
2nd commonest cause of nephrotic syndrome in adults
Can be primary or secondary
Renal biopsy shows sub-epithelial immune complex deposition in basement membrane
Treat with steroids etc
30% will progress to ESRF
Important secondary causes of membranous nephropathy
Infectious - Hep B and parasites
Connective tissue disease e.g. lupus
What is IgA Nephropathy
Most common GN in the world
Asymptomatic micro haematuria +/- non-nephrotic range proteinuria
Post-infection (GI/Resp) macro haematuria
Can cause AKI/CKD
Associated with Henoch-Schonlein Purpura
Renal Biopsy Shows mesangial cell proliferation nd expansion on LM with IgA deposits in mesangium on IF
25% progress to ESRF
What type of GN presents with arthritis/colitis/purpuric skin rash?
These are classic of the Henoch-Schonlein Purpura (HSP)
What is Rapidly progressive glomerulonephritis
A treatable cause of acute renal failure with rapid deterioration in renal function over a few days/weeks which may be part of a systemic disease
It is associated with glomerular crescents on biopsy and active urinary sediment (RBCs and granular casts)
ANCA-positive causes of RPGN
ANCA-negative causes of RPGN
Goodpastures disease (Anti-GBM)
Treatment of RPGN
Treatment should be prompt and consist of both supportive and immunosuppression care (and dialysis if needed)
Steroids e.g. pred oral/ IV methylpred
Cytotoxics e.g (Cyclophosphamide/ Mycophenolate)
What are the primary causes of Nephrotic Syndrome
Main Primary causes of Nephritic Syndrome
Secondary causes of Nephrotic Syndrome
SLE - in vascular nephritis
Secondary causes of Nephritic Syndrome
SLE - in other forms of nephritis
Anti-GBM/ Goodpastures Disease
What is a typical patient presentation for IgA Nephropathy?
Young man with episodic macroscopic haematuria
Recovers rapidly between attacks
Typical Patient presentation for Henloch-Sconlein Purpura
Purpuric rash on extensors - usually legs, abdo pain, fleeting poly-arthritis
Focal Segmental Glomerulosclerosis is the 2nd most common cause of Nephrotic in adults? true/false
It is the most common cause
2nd Commonest cause of Nephrotic syndrome in adults?