Nephritic Glomerulonephritis Flashcards
What is the most common primary glomerulonephritis?
IgA nephropathy
How does IgA nephropathy present?
Young patient (male)
- Asymptomatic non-visible haematuria
- OR Episodic visible haematuria which may be synpharyngitic aka within 12-72h of infection
What conditions is IgA nephropathy associated with?
- Alcoholic cirrhosis
- Coeliac disease/dermatitis herpetiformis
- Henoch-Schonlein purpura
What are symptoms of IgA nephropathy?
Asymptomatic or increased BP ± increased creatinine, proteinuria (usually < 1g)
What % of patients progress to renal failure?
20-50% (minority)
How do you diagnose IgA nephropathy?
Renal biopsy
What would you see on renal biopsy in IgA nephropathy?
- IgA deposition in mesangium
- Mesangial hypercellularity
- Positive immunofluorescence for IgA & C3
How do you treat IgA nephropathy and Henoch-Schonlein purpura?
- ACE-i/ARB (reduce proteinuria and protect renal function)
- Corticosteroids (good for gut involvement in HSP) + fish oil if proteinuria > 1g despite 3-6 months of ACE-i/ARB and GFR > 50
What happens in Henoch-Schonlein purpura?
- Small vessel vasculitis (systemic variant of IgA nephropathy)
- IgA deposition in kidney, skin, joints, gut
How do you diagnose Henoch-Schonlein purpura?
- Usually clinical diagnosis
- Confirm with positive immunofluorescence for IgA & C3 in skin
- Renal biopsy identical to IgA nephropathy
What is symptoms of Henoch-Schonlein purpura?
- Purpuric rash on extensor surface, typically legs
- Flitting polyarthritis
- Abdominal pain (GI bleeding)
- Nephritis
What is nephritis?
Haematuria due to inflammatory damage
What is nephrosis?
Proteinuria due to podocyte pathology
What investigations do you want to do in all glomerulonephritis?
- Bloods
- Immunoglobulins
- Electrophoresis
- C3 & C4
- ANA, ANCA, ds-DNA, anti-GBM (autoantibodies)
What imaging/further investigations do you want to do in all glomerulonephritis?
- CXR - pulmonary haemorrhage
- Renal US + biopsy
What happens in post-streptococcal glomerulonephritis?
Following throat (2 weeks) or skin infection (3-6 weeks), strep antigen deposits in glomerulus causing immune complex formation and inflammation
How does post-streptococcal glomerulonephritis present?
- Varies - haematuria or acute nephritis
- Oedema
- Raised BP
- Oliguria
How do you diagnose post-streptococcal glomerulonephritis?
- Evidence of streptococcal infection
- Increased ASOT (antistreptolysin O titre) - blood test that looks for antibodies to the streptococcus A bacteria
- Anti-DNAase B - antibody produced by the immune system in response to a strep infection
- Reduced serum levels of C3
How do you treat post-streptococcal glomerulonephritis?
- Supportive
- Antibiotics - to clear the nephritogenic bacteria
What is goodpasture’s syndrome now called?
Anti-GBM disease
What happens in anti-GBM disease?
Autoantibodies to type IV collagen present in glomerular and alveolar basement membranes
How does anti-GBM disease present?
- Renal disease (oligouria/anuria, haematuria, AKI, renal failure)
- Lung disease (pulmonary haemorrhage - SOB, haemoptysis)
How do you diagnose anti-GBM disease?
Anti-GBM in circulation/kidney (immunofluorescence for IgG)
How do you treat anti-GBM disease?
- Plasma exchange
- Corticosteroids
- Cyclophosphamide
What is rapidly progressive glomerulonephritis (crescentic GN)?
Any aggressive glomerulonephritis, rapidly progressing to renal failure in days or weeks
What are causes of rapidly progressive glomerulonephritis?
- Small vessel/ANCA vasculitis
- Lupus nephritis
- Anti-GBM disease
- Other GNs may transform to become rapid progressive incl. IgA, membranous
How do you diagnose rapidly progressive glomerulonephritis?
Crescents seen on renal biopsy (due to breaks in the GBM allowing an influx of inflammatory cells)
How do you treat rapidly progressive glomerulonephritis?
- Corticosteroids
- Cyclophosphamide
- Plasma exchange for anti-GBM/ANCA vasculitis
- ?Mabs in lupus nephritis
What indicates worse prognosis in anti-GBM disease?
- Dialysis dependence at presentation
- Increased crescents seen on biopsy
