Nephritic syndrome Flashcards
Define nephritic syndrome
Clinical syndrome that results in:
- Haematuria
- Oedema
- Elevated blood pressure
- Decreased urine output
What are the features of nephritic syndrome
- Proteinuria (< nephrotic)
- Haematuria (cola coloured)
- Azootemia (elevated urea and creatinine)
- Red cell clasts
- Oliguria
- Hypertension
What are the causes of nephritic syndrome
Acute glomerulonephritis (post-infectious)
IgA nephropathy (Berger) - Most common worldwide
Benign familial glomerulonephritis/Thin basement membrane)
Membranoprolfierative glomerulonephritis
Crescenteric/rapidly progressive:
- Goodpasture’s
- Immune complex (vasculitis): henoch-schonlein P, IgA, SLE, Alport’s, post-strep
- Pauci (vasculitis): microscopic polyarteritis, granulomatosis with polyangitis (Wegner’s)
What causes post-infectious acute glomerulonephritis
Most often post-streptococcal (GAS/pyogenes), 7-14 days after
Immune complex (IgG, IgM and C3 deposition in the glomeruli)
What investigations should be done for acute glomerulonephritis
Anti-Streptolysin O (ASO) titre: raised
Anti-DNAse B titres: raised
Complement: Low C3 (returns to normal in 3-4 weeks)
Renal biopsy:
- Increased cellularity of glomeruli - endothelial proliferation with neutrophils
- Electron microscopy: subepithelial humps (complex deposits)
- Immunofluorescence: Granular IgG deposits/ “starry sky” appearance
What is the management for post-infectious glomerulonephritis and what is the prognosis
Supportive
Good long term prognosis
What causes IgA nephropathy and what is is associated with
Immune complex deposition in the glomeruli
Associated with: alcoholic cirrhosis | coeliac disease | dermatitis herpetiformis | henoch-schonlein purpura
How does IgA nephropathy present
1-2 days after URTI → painless macroscopic haematuria ± vasculitis rashes
What is the difference between IgA nephropathy and post-strep glomerulonephritis
Post-strep: low complement levels
Post-strep: main symptom is proteinuria rather than haematuria
Post-strep: larger interval between URTI and onset of renal problems
What do investigations show for IgA nephropathy
IgA: Raised
Biopsy: deposits in mesangium
What is the management for IgA nephropathy
Isolated haematuria + minimal proteunuria + normal GFR → supportive and follow up
Persistent proteinuria → ACEi
Active disease (falling GFR) → immunosuppression with corticosteroids
What is the prognosis for IgA nephropathy
1/3 asymptomatic
1/3 CKD
1/3 progressive CKD → requires dialysis
Markers of poor prognosis: male gender, proteinuria (especially > 2 g/day), hypertension, smoking, hyperlipidaemia, ACE genotype DD
What are the features of the crescenteric glomerulonephritides
Rapidly progressive → ESRF within weeks
Formation of epithelial crescent seen the majority of the glomeruli
Crescents can be seen on light microscopy
Describe goodpasture’s disease (cause, features, fluorescence microscopy appearance, management)
Type II hypersensitivity: Anti-GBM antibodies (anti-T4 collagen Abs)
Causes nephritic and lung (pulmonary haemorrhage → haemoptysis) pathology
fluorescence microscopy: linear deposition
Management: Plasmapheresis + prednisolone + cyclophosphamide
- Prophylactic trimethoprim- for early phases of treatment due to immunosuppression
Which nephritic syndromes are immune-complex mediated and what is the appearance on fluorescence microscopy
Post-streptococcal glomerulonephritis
IgA nephropathy
SLE
Alport’s
HSP
Fluorescence: granular deposition
