Glomerular Disease

Question 1

What is glomerulonephritis?

Answer 1

Inflammation of the glomerulus
Responsible for 15% end stage kidney disease

Question 2

How is glomerulonephritis diagnosed?

Answer 2

Often relies on kidney biopsy and classified following findings

Question 3

What are features of glomerulonephritis?

Answer 3

Harmaturia
Proteinurea
Hypertension
Renal impairment

Question 4

What is a nephritic state?

Answer 4

Active urine sediment dysmorphic rbcs cellular casts
Hypertension
Renal impairment

Question 5

Nephrotic syndrome

Answer 5

Odema
Nephrotic range proteinuria:3.5g per day or 350mg/mmol creatinine
Hypoalbuminemia
Dyslipidaemia

Question 6

What can glomerulonephritis be separated into?

Answer 6

Proliferation and non proliferation

Question 7

What is proliferation glomerulonephritis?

Answer 7

Excessive numbers of cells in glomeruli these include infiltrating leukocytes

Question 8

Non proliferative glomerulonephritis?

Answer 8

Glomeruli look normal or have areas of scarring
They have normal numbers of cells

Question 9

Diffuse

Answer 9

> 50% glomeruli affected

Question 10

Focal

Answer 10

<50% glomeruli affected

Question 11

Global

Answer 11

All glomeruli affected

Question 12

Segmental

Answer 12

Part of the glomerulus affected

Question 13

What is the most common cause of glomerulonephritis?

Answer 13

IgA nephropathy

Question 14

What is IgA nephropathy characterised by?

Answer 14

IgA deposition in mesangium and mesangial proliferation

Question 15

When is IgA nephropathy most common?

Answer 15

In 2nd and 3rd decades of life

Question 16

What is the presentation of IgA nephropathy?

Answer 16

Microscopic haematuria
Microscopic haematuria and proteinuria
Nephrotic syndrome
IgA crescentic glomerulonephritis

Question 17

What is post streptococcal glomerulonephritis?

Answer 17

Follows 10-21 days after infection typically throat or skin
Most commonly with lancefeild group A streptococci

Genetic predisposition HLA-DR -DP

Question 18

What is Anti GBM disease?

Answer 18

Rare disease caused by circulating anti GBM

Around 10-20% of crecentix glomerulonephritis

Question 19

How does anti GMB disease present?

Answer 19

Nephritis
Nephritis lung haemorrhage

Question 20

How is Anti GBM disease diagnosed?

Answer 20

Two peaks 3rd decade and and 6/7th decade of life
Diagnosed by demonstrating anti-GBM antibodies in serum and kidney

Question 21

What is treatment for anti-GBM disease?

Answer 21

Aggressive immune suppression steroid plasma exchange and chclophophamide

Question 22

Summarise proliferative glomerulonephritides?

Answer 22

Present with nephritic state
Haematuria
Variable proteinuria
Hypertension
Renal impatiente
Rapid decline in function that leads to dialysis
Early diagnosis necessary to save nephrons

Question 23

Non proliferative glomerulonephritis?

Answer 23

Minimal change disease
Focal and segmental glomerulonephritis
Membranous nephropathy

Question 24

Nephrotic syndrome management?

Answer 24

treat oedema: salt and fluid restriction and loop diuretics
Hypertension use renin angiotensisn aldosterone blockade
Reduce risk of thrombosis heparin or warfarin
Reduce infective risk pneumococcal vaccine
Treat dyipidaemia

Specific therapy towards cause of non proliferative glomerulonephritis

Question 25

Minimal change disease?

Answer 25

Commonest form in children
Sudden onset odema
Remission with steroids
Relapse in two thirds of patients

Question 26

Minimal change disease treatment

Answer 26

Prednisone
Once remission achieved slow taper over 6 months
Initial pelapse treated with steroid course

Question 27

What do subsequent relapses of minimal change disease get treated with?

Answer 27

Cuclophophamide
Cyclosporine
Tacrilimus
Mycophenolaye
Rituximab

Question 28

Membranous nephropathy

Answer 28

Commonest cause nephrotic syndrome adults
Majority cases occur in isolation

Serological markers
Antiphospholipidase a2 receptor
Theombospondun

Secondary causes
Malignancies
Ale
Rheumatoid arthritis
Drugs

Question 29

How is membranous nephropathy treated

Answer 29

General measure for at least 6 months
Immunosuppressive
Cyclophosphamide and steroids for 6 months
Tacrilimus
Rutuximab

Resolves spontaneously in a third of patients

Question 30

30 male referred to the renal clinic by GP for incidental microscopic haematuria.
BP 150/90 mmHg.
Creatinine 80 µmol/L, eGFR>60 ml/min/1.73m2.
Urine dip: RBC+++, Protein ++.
Urine protein creatinine ratio=180mg/mmol creatinine
Renal US: normal kidney size with no structural abnormalities.

what do you do?

Answer 30

You decide to start ACE-I.
You also decide to perform a renal biopsy.
Renal biopsy confirms IgA nephropathy.

Question 31

30 male referred to the renal clinic by GP for incidental microscopic haematuria.
BP 150/90 mmHg.
Creatinine 80 µmol/L, eGFR>60 ml/min/1.73m2.
Urine dip: RBC+++, Protein ++.
Urine protein creatinine ratio=180mg/mmol creatinine
Renal US: normal kidney size with no structural abnormalities.

following initial treatment

At 5 years: serum creatinine 210 µmol/L and eGFR 32 ml/min/1.73m2.
10 years later, serum creatinine 480 µmol/L and eGFR 14 ml/min/1.73m2.
Brother considered as kidney donor for living kidney transplantation.

what is he suffering from?

Answer 31

Post Infectious Glomerulonephritis

Question 32

Six-year-old boy brought to GP generally unwell, dark urine

Puffy face, no rashes no oedema, BP 135/86mmHg

Sore throat 2 weeks previously

Presumptive diagnosis post-streptococcal nephritis

what investigations should be done?
how should this be treated?

Answer 32

Hb 130g/L; WCC 11.3 x 109.
Urea 11.2mmol/L, creatinine 160μmol/L.
Urine microscopy RBC +++, casts +.
Complement: low C3.

Antibiotics for infection, debatable.
Loop diuretics such as frusemide for oedema.
Anti-hypertensives e.g. vasodilator drugs.

Question 33

what are different types of crescentic glomerulonephritis?

Answer 33

Question 34

60-year-old woman presents to GP with three-month history of increasing tiredness, anorexia and weight loss.

Physical examination revealed a rash on both ankles and lower legs, and bilateral basal crepitations: BP 150/85 mmHg.

Urine dipstick: protein+++ and blood++.

Serum creatinine 640 µmol/L, eGFR 8 ml/min/1.73m2.

what investigations and treatment should be done?

Answer 34

Anti-neutrophil cytoplasmic antibodies positive with MPO-ANCA of >134 units/L.
Renal biopsy
Focal necrotizing glomerulonephritis.
Treatment:
High dose steroids
Cyclophosphamide
Plasma exchange

Question 35

22-year-old woman presents with bilateral leg oedema which has developed suddenly over a week.
She also describes breathless and chest pain.
Urine dip ++++ protein.

what investigations and treatment should be done?

Answer 35

Urine protein creatinine ration 400mg/mmol.
Creatinine 60 mmol/l and eGFR of >60 ml/min/1.73m2
Serum Albumin 15 g/L
Haemoglobin 154 g/L
Cholesterol 9.2 mmol/l
Pregnancy test negative.

Salt and fluid restrictions.
Daily weights.
Loop diuretic e.g. Furosemide.
Thromboprophylaxis e.g. low molecular weight heparin.

Prednisolone 60 mg per day
Achieved remission after 8 weeks of treatment.
Diuretics and thromboprophylaxis stopped.
Prednisolone dose tapered over 6 months.

Question 36

what is focal and segmental glmerulonephritis?

Answer 36

Is not a single disease, rather a syndrome with multiple causes.
Presents with nephrotic syndrome.
Pathology reveals focal and segmental sclerosis with distinctive patterns e.g.
tip lesion, collapsing, cellular, perihilar, and not otherwise specified.
Primary (idiopathic) or secondary.
Generally steroid resistant.
High chance of progression to end stage kidney disease.

Question 37

what is the treatment for focal and segmental glmerulonephritis?

Answer 37

General measures, as previously described.
Trail of steroids, positive response , even partial remission, carries better prognosis.
Alternative options: cyclosporin, cyclophosphamide, and Rituximab.

Question 38

34-year-old man presented with severe oedema which developed over 3 weeks

Investigations
Urine PCR 560mg/mmol creatinine
Serum albumin 18 g/L
Serum creatinine 130 µmol/L, eGFR 56 ml/min/1.73m2

Renal biopsy showed FSGS.

what is the treatment?

Answer 38

Diuretics helped to reduce oedema.
Prednisolone - no response.
Trial of cyclosporin – progressively rising serum creatinine.
Serum creatinine at 280 µmol/L and eGFR 26 ml/min/1.73m2 at 8 months from presentation.
At 18 months serum creatinine 540 µmol/l and eGFR 11 ml/min/1.73m2.
Pre-emptive renal transplantation from brother, early recurrence post-transplant.
Plasma exchange and Rituximab.
Remission at 3 months post-transplant with serum creatinine 80µmol/L and normal serum albumin.

Question 39

66-year old man presented with cough, breathlessness, and peripheral oedema.
He smokes 20 cpd.
Urine dip: Proteinuria ++++
Urine protein creatinine ration 580mg/mmol.
Serum albumin 20 g/L.
Serum creatinine 135mmol/l
Renal biopsy: confirms membranous nephropathy.

what investigations and treatment should be done?

Answer 39

CT chest/abdomen/pelvis: suggestive of lung cancer.
Bronchoscopy and biopsy confirm squamous cell carcinoma.
General measures for nephrotic syndrome.
No immunesuppression.
At 3 months: partial remission following radiotherapy
Present with nephrotic syndrome.
Renal biopsy is key investigation.
Identify cause, if possible.
General measures are important in all cases.
Specific treatment as appropriate.