Nephritic syndrome Flashcards

1
Q

Define nephritic syndrome

A

Clinical syndrome that results in:
- Haematuria
- Oedema
- Elevated blood pressure
- Decreased urine output

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2
Q

What are the causes of nephritic syndrome

A

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)

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3
Q

What are the features of nephritic syndrome

A
  • Proteinuria (< nephrotic)
  • Haematuria (cola coloured)
  • Azootemia (elevated urea and creatinine)
  • Red cell clasts
  • Oliguria
  • Hypertension
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4
Q

What causes post-infectious acute glomerulonephritis

A

Most often post-streptococcal (GAS/pyogenes), 7-14 days after
Immune complex (IgG, IgM and C3 deposition in the glomeruli)

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5
Q

What investigations should be done for acute glomerulonephritis

A

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

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6
Q

What is the management for post-infectious glomerulonephritis and what is the prognosis

A

Supportive
Good long term prognosis

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7
Q

What causes IgA nephropathy and what is is associated with

A

Immune complex deposition in the glomeruli

Associated with: alcoholic cirrhosis | coeliac disease | dermatitis herpetiformis | henoch-schonlein purpura

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8
Q

How does IgA nephropathy present

A

1-2 days after URTI → painless macroscopic haematuria ± vasculitis rashes

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9
Q

What is the difference between IgA nephropathy and post-strep glomerulonephritis

A

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

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10
Q

What do investigations show for IgA nephropathy

A

IgA: Raised
Biopsy: deposits in mesangium

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11
Q

What is the management for IgA nephropathy

A

Isolated haematuria + minimal proteunuria + normal GFR → supportive and follow up
Persistent proteinuria → ACEi
Active disease (falling GFR) → immunosuppression with corticosteroids

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12
Q

What is the prognosis for IgA nephropathy

A

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

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13
Q

What are the features of the crescenteric glomerulonephritides

A

Rapidly progressive → ESRF within weeks
Formation of epithelial crescent seen the majority of the glomeruli
Crescents can be seen on light microscopy

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14
Q

Describe goodpasture’s disease (cause, features, fluorescence microscopy appearance, management)

A

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

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15
Q

Which nephritic syndromes are immune-complex mediated and what is the appearance on fluorescence microscopy

A

Post-streptococcal glomerulonephritis
IgA nephropathy
SLE
Alport’s
HSP

Fluorescence: granular deposition

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16
Q

What is the management for immune-complex mediated nephritic syndrome

A

Immune Complex (not SLE)
- Prednisolone
- Phenoxymethylpenicillin

SLE
Cyclophosphamide +/- prednisolone
Prophylactic trimethoprim- for early phases of treatment due to immunosuppression

17
Q

Which nephritic syndromes are Pauci immune, what is seen on fluorescence microscopy, and what is the management

A

pANCA → microscopic polyangiitis
cANCA → Granulomatosis with polyangiitis (Wegner’s)

FM: scanty/lack of deposition

Management:
Methyl prednisolone + cyclophosphamide
Prophylactic trimethoprim- for early phases of treatment due to immunosuppression

18
Q

What is Alport’s syndrome, how does it present, and how is it managed

A

X-linked
Presents at 5-20yo with end stage renal failure
Impacts type IV collagen alpha-5 chain
1. Haematuria
2. Cataracts
3. Sensorineural defects

Mx: dialysis

19
Q

Describe benign familial/thin basement membrane

A

Autosomal dominant
Impacts collagen IV alpha-4
Presents with painless haematuria