Nephrotic and Nephritic Syndrome



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Nephrotic and Nephritic Syndrome

  • Dr Claire Gibbons
  • FY2
  • Claire.gibbons@doctors.org.uk

Learning Objectives

  • Understand and define nephrotic and nephritic syndromes.
  • Describe the initial investigations and management of nephrotic and nephritic syndromes.
  • Describe the complications of nephrotic and nephritic syndromes.
  • Draw a nephron!

Glomerulonephritis

  • Glomerulus – capillary loop with basement membrane which allows passage of specific molecules into the nephron
  • Glomerulonephritis – inflammation/damage of the glomerular basement membrane resulting in altered function. Relatively uncommon cause of kidney injury.
  • Can present as nephrotic and/or nephritic syndrome.

What is nephrotic syndrome?

  • Increased permeability of the glomerulus leading to loss of proteins into the tubules.

Nephrotic Syndrome

  • Triad of:
    • MASSIVE Proteinuria >3g/24hours
      • Or spot urine protein:creatinine ratio >300-350mg/mmol
    • Hypoalbuminaema <25g/L
    • Oedema
  • And often:
    • Hypercholesterolaemia/dyslipidaemia (total cholesterol >10mmol/L)

Presentation

  • New-onset oedema
    • Initially periorbital or peripheral
    • Later genitals, ascites, anasarca
  • Frothy urine
  • Generalised symptoms – lethargy, fatigue, reduced appetite

Further possible presentations...

  • Oedema
  • BP normal/raised
  • Leukonychia
  • Breathlessness:
    • Pleural effusion, fluid overload, AKI
  • DVT/PE/MI
  • Eruptive xanthomata/ xanthalosmata

You are a GP with the following patients...

  • Young, fit 24 year old male complaining of frothy urine.
  • 10 year old boy with puffy eyes.
  • 74 year old female with multiple co-morbidities and swollen ankles.

Differential Diagnosis for Oedema

  • Congestive Cardiac Failure
    • Raised JVP, pulmonary oedema, mild proteinuria
  • Liver disease
    • Hypoalbuminaemia, ascites/oedema
  • What investigations can you do?
  • You decide to send your patient to the renal clinic...

Causes of Nephrotic Syndrome

  • Primary glomerulonephritis
    • Minimal change disease (80% paeds cases)
    • Focal segmental glomerulosclerosis (most common cause in adults)
    • Membranous glomerulonephritis

Systemic Causes

  • Secondary glomerulonephritis
    • Diabetic nephropathy
    • Sarcoidosis
    • Autoimmune: SLE, Sjogrens
    • Infection: Syphilis, hepatitis B, HIV
    • Amyloidosis
    • Multiple myeloma
    • Vasculitis
    • Cancer
    • Drugs: gold, penicillamine, captopril, NSAIDs

Investigations

  • Urine dipstick and send to lab
  • Urine microscopy
  • Bloods – the usual ones, plus renal screen
    • Immunoglobulins, electrophoresis (myeloma screen), complement (C3, C4) autoantibodies (ANA, ANCA, anti-dsDNA, anti-GBM)
  • Renal ultrasound
  • Renal biopsy (all adults)
    • Children generally trial of steroids first

Management

  • Conservative
    • Monitor U&E, BP, fluid balance, weight
    • Salt and fluid restriction
    • Treat underlying cause
  • Medical
    • Diuretics
    • ACE-inhibitors/ARBs
    • Corticosteroids/immunosuppression
    • Dialysis
    • Anticoagulation
  • Surgical
    • Renal transplant

Complications

  • Increased susceptibility to infection
    • 20% adult cases
    • Due to reduced serum IgG, reduced complement activity, reduced T cell function
  • Thromboembolism
    • 40% adult cases
    • Partly due to increased clotting factors and platelet abnormalities
  • Hyperlipidaemia

Prognosis

  • Varies
  • With treatment, generally good prognosis
    • Especially minimal change disease (1% progress to ESRF)
  • Without treatment, very poor prognosis
    • Children under 5 or adults older than 30 = worse prognosis
  • What is nephritic syndrome?

Pathophysiology

  • Thin glomerular basement membrane with pores that allow protein and blood into the tubule.

Nephritic Syndrome

  • Clinical syndrome defined by:
    • Haematuria/ red cell casts
    • Hypertension (mild)
    • Oliguria
    • Uraemia
    • Proteinuria (<3g/24 hours)

Signs and Symptoms

  • Haematuria (E.g. cola coloured)
  • Proteinuria
  • Hypertension
  • Oliguria
  • Flank pain
  • General systemic symptoms
  • Post-infectious = 2-3 weeks after strep-throat/URTI

What are your differentials?

  • Malignancy (older patients)
  • UTI
  • Trauma
  • What bedside investigation would you like to do?
  • You decide to refer to the renal clinic...

Causes

  • Post-infectious glomerulonephritis
  • Primary
    • IgA Nephropathy (Berger's disease)
    • Rapidly progressive glomerulonephritis
    • Proliferative glomerulonephritis
  • Secondary glomerulonephritis
    • Henoch-Schonlein purpura
    • Vasculitis

Investigations

  • Urine dipstick and send sample to lab
  • Urine microscopy – red cell casts
  • Bloods – the usual plus renal screen
    • Immunoglobulins, electrophoresis, complement (C3, C4) autoantibodies (ANA, ANCA, anti-dsDNA, anti-GBM); blood culture; ASOT (anti-streptolysin O titre)
  • Renal ultrasound
  • Renal biopsy

Red Cell Casts

Management

  • Conservative
    • Monitor U&E, BP, fluid balance, weight
    • Salt and fluid restriction
    • Treat underlying cause
  • Medical
    • Diuretics
    • Treat hypertension
    • Corticosteroids/immunosuppression
    • Dialysis
  • Surgical
    • Renal transplant

Prognosis

  • Varies
  • Post-infectious usually self-resolving (95% recover renal function)
  • Others are a bit more nasty

Example Case

Summary

  • Nephrotic syndrome = MASSIVE proteinuria
  • Nephritic syndrome = haematuria/red cell casts
  • May be a mixed presentation
  • New oedema? Dipstick that urine!
  • Haematuria? Exclude malignancy!
  • Any Questions?
  • Claire.gibbons@doctors.org.uk

Sources

  • Oxford Handbook of Clinical Medicine
  • Oxford Handbook for the Foundation Programme
  • Essential Revision Notes for the MRCP
  • Www.almostadoctor.com
  • Www.pathologystudent.com


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