Nephritic Sx

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Nephritic Sx Nephrotic Sx
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Case report 1

• 18 yr old man
• Bilateral loin pain
• Macroscopic haematuria
• Sore throat started one day earlier
• BP 140/90 euvolaemic
• Creatinine 120 µmol/l
• Proteinuria and haematuria on dipstix

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Case Report 2

• 20 yr old lady
• Completely well
• Haematuria on dipstix
• No proteinuria
• Normotensive

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Case Report 3

• 12 year old boy
• Impetigo two weeks earlier
• Headache
• Oliguric
• Frothy dark coloured urine
• Hypertensive

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Case report 4

• 15yr old woman
• 3/12 ankle swelling face and fingers swollen in
  the am
• BP 130/80 JVP normal Leg oedema
• Creatinine 54 µmol/l
• Cr Cl 140 ml/min
• Albumin 18 g/l
• 24 hr u.protein 10 g

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Case Report 5

• 30 year old man,diabetic
• Known hypertensive
• Ankle oedema
• Dipstix proteinuria
• Creatinine 124 µmol/l (80 120)
• Albumin 30 g/l (36 45)

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Case Report 6

• 50 year old obese man
• Hypertension 10 years
• NIDDM 3 years
• No retinopathy
• Creatinine 124 µmol/l
• 24 hr urine protein 2 g
• HbA1 9.6

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Structure of the filtration barrier
Podocyte Foot processes Fenestrated endothelium
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Minimal change disease
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Glomerular changes in disease

• Proliferation
• Sclerosis
• Necrosis
• Increase in mesangial matrix
• Changes to basement membrane
• Immune deposits
• Diffuse vs focal
• Global vs segmental

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Common Syndromes

• Nephrotic Syndrome
• Nephritic Syndrome
• Rapidly Progressive GN
• Loin Pain Haematuria Syndrome

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Features of Glomerular Disease

• Proteinuria
• Haematuria
• Renal Failure
• Salt and Water Retention
• Loin Pain

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Salt and Water Retention

• Hypertension
• Oedema
• Oliguria

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Loin Pain

• Rare

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Proteinuria

• Marker of renal disease
• Risk factor for cardiovascular disease
• Dyslipidaemia
• Hypertension
• Something more?
• 24 hr protein vs urine proteincreatinine ratio

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Nephrotic syndrome

• Proteinuria gt 40 mg/m2hr
• Hypoalbuminaemia (lt2.5mg/dl)
• Oedema
• Hyperlipidemia
• Thromboses
• Infection

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Learning Points

• Clinical features
• Commonest types
• Prognosis
• Causes
• Treatments

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Nephrotic Syndrome

• Causes of primary idiopathic NS
• Minimal change disease
• Mesangial proliferation
• Focal segmental glomerulosclerosis

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Minimal Change Disease

• Usually children
• Nephrotic syndrome with highly selective
  proteinuria and generalised oedema
• Rarely hypertension or ARF
• T cell mediated VPF
• Steroid sensitive usually
• Spectrum of disease to FSGS

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Focal Segmental Glomerulosclerosis

• Juxtamedullary glomeruli may be missed due to
  sampling error
• Older patients
• Less sensitive to immunosuppression
• Hypertension, haematuria, progressive CRF

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FSGS

• Familial
• VUR
• Drug abuse
• Obesity

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Common types of GN

• Primary
• Thin membrane disease
• IgA disease
• Minimal Change / FSGS spectrum
• Membanous Nephropathy
• Secondary
• PSGN Diabetic Glomerulosclerosis

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Rarer Types

• Diffuse endocapillary proliferative GN (post
  infectious GN)
• Crescentic GN
• Membanoproliferative / mesangiocapillary GN

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Nephritic Syndrome

• Haematuria
• Hypertension
• Oliguria
• Edema

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Rapidly progressive GN

• Nephritic or nephrotic onset
• ESRF in six months

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General Treatment of GN

• Control BP
• Angiotensin blockade
• Statin
• Lose weight
• Stop smoking
• (pneumococcal prophylaxis)
• (anticoagulation)

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Help!

• I need a volunteer!

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Case report 1

• 18 yr old man
• Bilateral loin pain
• Macroscopic haematuria
• Sore throat started one day earlier
• BP 140/90 euvolaemic
• Creatinine 120 µmol/l
• Proteinuria and haematuria on dipstix

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Case 1 indicative answers

• IgA Disease
• Renal failure, proteinuria, haematuria, oedema,
  hypertension, oliguria, loin pain
• All except oedema and oliguria

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Mesangial IgA disease

• Classical Bergers Disease
• Microscopic haematuria
• Proteinuria (rarely nephrotic)
• Hypertension
• Chronic renal failure
• ? Failure of hepatic clearance of IgA
• Association with GI disease
• No specific treatment

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Ig A Nephropathy

• Ig A nephropathy is the most common primary GN
  worldwide
• Usually present with hematuria
• Episodes of gross hematuria are precipitated by
  flu like illness, exercise
• Urinary protein excretion usually non-nephrotic
• Associated with chronic liver ds, psoriasis, IBD
  and HIV disease.

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Ig A Nephropathy

• Only 30 of patients with IgA nephropathy has
  progressive disease.
• In progressive disease, use of fish oil may be
  beneficial.
• Immunosuppressive therapy in patients with Ig A
  nephropathy has not consistently shown to be of
  benefit

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Case Report 2

• 20 yr old lady
• Completely well
• Haematuria on dipstix
• No proteinuria
• Normotensive

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Case 2 indicative answers

• Exclude menstruation!
• Thin membrane disease (possibly IgA disease)
• Commonest cause of isolated microscopic
  haematuria in this age group.
• At this age, urological cause unlikely nil to
  suggest infection / urolithiasis

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Thin membrane disease

• Most common GN
• Microscopic haematuria
• Familial
• Benign
• No treatment needed
• Most young people with isolated microscopic
  haematuria have thin membrane disease

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Case Report 3

• 12 year old boy
• Impetigo two weeks earlier
• Headache
• Oliguric
• Frothy dark coloured urine
• Hypertensive

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Case 3 indicative answers

• Acute nephritic syndrome
• Post-streptococcal glomerulonephritis
• Diffuse proliferative endocapillary
  glomerulonephritis
• Due to salt and water retention, so salt
  restriction or loop diuretic

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Acute Post-Infectious GN

• Usually occur in children
• Post-streptococcal GN is the most common cause of
  post infectious GN
• Occurs after a streptococcal sore throat or
  impetigo
• Caused by Group A, beta-hemolytic streptococci,
  particularly nephritogenic strains Type 1,4,12
  (throat) and 2,49(skin)

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Acute Post-Infectious GN

• Acute onset of gross hematuria (COLA COLORED) or
  microscopic hematuria after latent period of
  10-14 days.
• Edema/hypertension
• RBC casts on U/A
• Elevated creatinine, increased ASO titer
• Decreased complement level

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Acute Post-Infectious GN

• LM Diffuse proliferative and exudative GN
• IF IgG and C3 lumpy, bumpy
• EM Sub epithelial Hump or Flame like
  deposits

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Diffuse Endocapillary Proliferative GN (Post
Streptococcal GN)

• Diffuse endocapillary proliferative GN
• Post infectious usually Gp A Strep
• Acute nephritic syndrome
• Uraemia rare
• Self-limited rarely death from ?BP
• Abnormal RUA for up to 2 yrs
• Circulating immune complex mediated

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Acute Post-Infectious GN

• Renal biopsy is generally not required.
• Treatment is supportive and consist of sodium
  restriction, control of BP and dialysis if this
  become necessary.

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Complications of the Nephritic Syndrome

• Hypertensive encephalopathy (seizures, coma)
• Heart Failure (pulmonary oedema)
• Uraemia requiring dialysis

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Prognosis in the Nephritic Syndrome

• More than 95 of children make a complete
  recovery
• Chronic renal impairment in the longer term is
  uncommon in children
• Bad prognostic features include severe renal
  impairment at presentation and continuing heavy
  proteinuria and hypertension
• Adults more likely to have long term sequellae
  than children

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Case report 4

• 15 yr old girl
• 3/12 ankle swelling face and fingers swollen in
  the am
• BP 130/80 JVP normal Leg oedema
• Creatinine 54 µmol/l
• Cr Cl 140 ml/min
• Albumin 18 g/l
• 24 hr u.protein 10 g

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Case 4 indicative answers

• Minimal change focal segmental
  glomerulosclerosis spectrum
• Very nephrotic
• Age and borderline BP make FSGS more likely than
  MCN
• Effect of loss of colloid osmotic pressure
  gradient across glomerulus causing
  hyperfiltration

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Case Report 5

• 30year old man,diabetic
• Known hypertensive
• Ankle oedema
• Dipstix proteinuria
• Creatinine 124 µmol/l (80 120)
• Albumin 30 g/l (36 45)

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Case 5 indicative answers

• Nephrotic syndrome secondary to diabetes /
  membranous disease
• Refer urgently to nephrology

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Diabetic glomerulosclerosis

• Retinopathy
• Hypertension
• Microalbuminuria
• Nephrotic syndrome
• Renal failure usually progressive
• Poor prognosis on RRT

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What wed like!

• Demography including tel no and occupation
• Reason for referral presenting complaint,
  expectations
• Co-morbidities, incl other diagnoses, smoking,
  alcohol and BMI, social care needs
• Examination
• Medications (incl recently stopped), allergies
  etc
• Treatment and investigations to date
• Special requirements (eg interpreter)

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Case Report 6

• 50 year old obese man
• Hypertension 10 years
• NIDDM 3 years
• No retinopathy
• Creatinine 124 µmol/l
• 24 hr urine protein 2 g
• HbA1 9.6

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Case 6 indicative answers

• Obesity-related FSGS more likely than diabetic
  nephropathy (duration diabetes, absence of
  retinopathy)
• Worsening nephrotic syndrome and progressive
  renal failure Death from cardiovascular cause
  before reaches ESRF
• Stop smoking, lose weight, improve glycaemic
  control, regular exercise, healthy diet, moderate
  alcohol in that order

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Case 6 indicative answers contd

• Lack of ownership of responsibility for own
  health
• Withdrawal symptoms (smoking)
• Denial of calorie intake
• Difficulty exercising due to immobility
• No!
• Problems with MDRD equation
• No evidence of benefit of ACE inhibitors in
  absence proteinuria
• Dangers of ACE inhibitors in patients with
  angioneurotic oedema, hypotension or bilateral
  renal artery stenosis

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Lessons

• Not all abnormal urinalysis is a UTI
• Acute pyelonephritis is very rarely bilateral

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Haematuria Urologist or Nephrologist?

• Age
• Other features proteinuria etc
• Urine microscopy for casts
• Phase contrast microscopy

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Non-dysmorphic vs dysmorphic
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RBC Cast
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AntiGBM disease

• RPGN Lung haemorrhage
• Destructive process medical emergency!
• Antibody-mediated
• One hit
• High dose immunosuppression
• Plasma exchange

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• Any Questions?

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