Nephritic Sx & Nephrotic Sx

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

Case Report 2

20 yr old ladyCompletely wellHaematuria on dipstixNo proteinuriaNormotensive

Case Report 3

12 year old boyImpetigo two weeks earlierHeadacheOliguricFrothy dark coloured urineHypertensive

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

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)

Case Report 6

50 year old obese manHypertension 10 yearsNIDDM 3 yearsNo retinopathyCreatinine 124 μmol/l24 hr urine protein 2 gHbA1 9.6%

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Structure of the filtration barrier

Podocyte

Foot processes

Fenestrated endothelium

Minimal change disease

Glomerular changes in diseaseProliferationSclerosisNecrosis Increase in mesangial

matrixChanges to basement

membrane Immune depositsDiffuse vs focalGlobal vs segmental

Common Syndromes

Nephrotic Syndrome

Nephritic Syndrome

Rapidly Progressive GN

Loin Pain Haematuria Syndrome

Features of Glomerular Disease

ProteinuriaHaematuriaRenal FailureSalt and Water RetentionLoin Pain

Salt and Water Retention

Hypertension

Oedema

Oliguria

Loin Pain

Rare

Proteinuria

Marker of renal disease Risk factor for

cardiovascular disease– Dyslipidaemia

– Hypertension

– Something more?

24 hr protein vs urine protein:creatinine ratio

Nephrotic syndrome

Proteinuria > 40 mg/m2*hrHypoalbuminaemia (<2.5mg/dl)OedemaHyperlipidemia

ThrombosesInfection

Learning Points

Clinical features Commonest typesPrognosisCauses Treatments

Nephrotic Syndrome

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

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

Focal Segmental Glomerulosclerosis

Juxtamedullary glomeruli – may be missed due to sampling error

Older patients Less sensitive to

immunosuppression Hypertension,

haematuria, progressive CRF

FSGS:

Familial

VUR

Drug abuse

Obesity

Common types of GN

PrimaryThin membrane diseaseIgA diseaseMinimal Change / FSGS spectrumMembanous Nephropathy

SecondaryPSGN & Diabetic Glomerulosclerosis

Rarer Types

Diffuse endocapillary proliferative GN (post infectious GN)

Crescentic GNMembanoproliferative / mesangiocapillary

GN

Nephritic Syndrome

Haematuria

Hypertension

Oliguria

Edema

Rapidly progressive GN

Nephritic or nephrotic onset

ESRF in six months

General Treatment of GN

Control BPAngiotensin blockadeStatinLose weightStop smoking(pneumococcal prophylaxis)(anticoagulation)

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

Case 1: indicative answers

IgA Disease

Renal failure, proteinuria, haematuria, oedema, hypertension, oliguria, loin pain

All except oedema and oliguria

Mesangial IgA disease

Classical Berger’s Disease Microscopic haematuria Proteinuria (rarely

nephrotic) Hypertension Chronic renal failure ? Failure of hepatic

clearance of IgA Association with GI

disease No specific treatment

Ig A Nephropathy

Ig A nephropathy is the most common primary GN worldwide

Usually present with hematuriaEpisodes of gross hematuria are precipitated by

flu like illness, exerciseUrinary protein excretion usually non-nephroticAssociated with chronic liver ds, psoriasis, IBD

and HIV disease.

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

Case Report 2

20 yr old ladyCompletely wellHaematuria on dipstixNo proteinuriaNormotensive

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

Thin membrane disease

Most common GNMicroscopic haematuriaFamilialBenignNo treatment neededMost young people with

isolated microscopic haematuria have thin membrane disease

Case Report 3

12 year old boyImpetigo two weeks earlierHeadacheOliguricFrothy dark coloured urineHypertensive

Case 3: indicative answers

Acute nephritic syndromePost-streptococcal glomerulonephritisDiffuse proliferative endocapillary

glomerulonephritisDue to salt and water retention, so salt

restriction or loop diuretic

Acute Post-Infectious GN

Usually occur in childrenPost-streptococcal GN is the most common cause of

post infectious GNOccurs after a streptococcal sore throat or impetigoCaused by Group A, beta-hemolytic streptococci,

particularly nephritogenic strains – Type 1,4,12 (throat) and 2,49(skin)

Acute Post-Infectious GN

Acute onset of gross hematuria (COLA COLORED) or microscopic hematuria after latent period of 10-14 days.

Edema/hypertensionRBC casts on U/AElevated creatinine, increased ASO titerDecreased complement level

Acute Post-Infectious GN

LM – Diffuse proliferative and exudative GN

IF – IgG and C3 “lumpy, bumpy”

EM – Sub epithelial “Hump” or “Flame” like deposits

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

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.

Complications of the Nephritic Syndrome

Hypertensive encephalopathy (seizures, coma)

Heart Failure (pulmonary oedema)

Uraemia requiring dialysis

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

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

Case 4: indicative answers

Minimal change – focal segmental glomerulosclerosis spectrum

Very nephrotic Age and borderline BP make FSGS more

likely than MCNEffect of loss of colloid osmotic pressure

gradient across glomerulus causing hyperfiltration

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)

Case 5: indicative answers

Nephrotic syndrome secondary to diabetes / membranous disease

Refer urgently to nephrology

Diabetic glomerulosclerosis

RetinopathyHypertensionMicroalbuminuriaNephrotic syndromeRenal failure –

usually progressivePoor prognosis on

RRT

What we’d like!

Demography including tel no and occupationReason for referral: presenting complaint,

expectationsCo-morbidities, incl other diagnoses, smoking,

alcohol and BMI, social care needsExaminationMedications (incl recently stopped), allergies etcTreatment and investigations to dateSpecial requirements (eg interpreter)

Case Report 6

50 year old obese manHypertension 10 yearsNIDDM 3 yearsNo retinopathyCreatinine 124 μmol/l24 hr urine protein 2 gHbA1 9.6%

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

Case 6: indicative answers contd

Lack of ownership of responsibility for own healthWithdrawal symptoms (smoking) Denial of calorie intakeDifficulty exercising due to immobilityNo!

– 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

Lessons

Not all abnormal urinalysis is a UTI

Acute pyelonephritis is very rarely bilateral

Haematuria Urologist or Nephrologist?

AgeOther features –

proteinuria etcUrine microscopy for

castsPhase contrast

microscopy

Non-dysmorphic vs dysmorphic

RBC Cast

AntiGBM disease

RPGN + Lung haemorrhage

Destructive process – medical emergency!

Antibody-mediatedOne hitHigh dose

immunosuppressionPlasma exchange

Any Questions?

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