Glomerular Disease Flashcards
What is glomerulonephritis?
Inflammation of the glomerulus
Responsible for 15% end stage kidney disease
How is glomerulonephritis diagnosed?
Often relies on kidney biopsy and classified following findings
What are features of glomerulonephritis?
Harmaturia
Proteinurea
Hypertension
Renal impairment
What is a nephritic state?
Active urine sediment dysmorphic rbcs cellular casts
Hypertension
Renal impairment
Nephrotic syndrome
Odema
Nephrotic range proteinuria:3.5g per day or 350mg/mmol creatinine
Hypoalbuminemia
Dyslipidaemia
What can glomerulonephritis be separated into?
Proliferation and non proliferation
What is proliferation glomerulonephritis?
Excessive numbers of cells in glomeruli these include infiltrating leukocytes
Non proliferative glomerulonephritis?
Glomeruli look normal or have areas of scarring
They have normal numbers of cells
Diffuse
> 50% glomeruli affected
Focal
<50% glomeruli affected
Global
All glomeruli affected
Segmental
Part of the glomerulus affected
What is the most common cause of glomerulonephritis?
IgA nephropathy
What is IgA nephropathy characterised by?
IgA deposition in mesangium and mesangial proliferation
When is IgA nephropathy most common?
In 2nd and 3rd decades of life
What is the presentation of IgA nephropathy?
Microscopic haematuria
Microscopic haematuria and proteinuria
Nephrotic syndrome
IgA crescentic glomerulonephritis
What is post streptococcal glomerulonephritis?
Follows 10-21 days after infection typically throat or skin
Most commonly with lancefeild group A streptococci
Genetic predisposition HLA-DR -DP
What is Anti GBM disease?
Rare disease caused by circulating anti GBM
Around 10-20% of crecentix glomerulonephritis
How does anti GMB disease present?
Nephritis
Nephritis lung haemorrhage
How is Anti GBM disease diagnosed?
Two peaks 3rd decade and and 6/7th decade of life
Diagnosed by demonstrating anti-GBM antibodies in serum and kidney
What is treatment for anti-GBM disease?
Aggressive immune suppression steroid plasma exchange and chclophophamide
Summarise proliferative glomerulonephritides?
Present with nephritic state
Haematuria
Variable proteinuria
Hypertension
Renal impatiente
Rapid decline in function that leads to dialysis
Early diagnosis necessary to save nephrons
Non proliferative glomerulonephritis?
Minimal change disease
Focal and segmental glomerulonephritis
Membranous nephropathy
Nephrotic syndrome management?
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
Minimal change disease?
Commonest form in children
Sudden onset odema
Remission with steroids
Relapse in two thirds of patients
Minimal change disease treatment
Prednisone
Once remission achieved slow taper over 6 months
Initial pelapse treated with steroid course
What do subsequent relapses of minimal change disease get treated with?
Cuclophophamide
Cyclosporine
Tacrilimus
Mycophenolaye
Rituximab
Membranous nephropathy
Commonest cause nephrotic syndrome adults
Majority cases occur in isolation
Serological markers
Antiphospholipidase a2 receptor
Theombospondun
Secondary causes
Malignancies
Ale
Rheumatoid arthritis
Drugs
How is membranous nephropathy treated
General measure for at least 6 months
Immunosuppressive
Cyclophosphamide and steroids for 6 months
Tacrilimus
Rutuximab
Resolves spontaneously in a third of patients
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?
You decide to start ACE-I.
You also decide to perform a renal biopsy.
Renal biopsy confirms IgA nephropathy.
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?
Post Infectious Glomerulonephritis
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?
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.
what are different types of crescentic glomerulonephritis?
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?
Anti-neutrophil cytoplasmic antibodies positive with MPO-ANCA of >134 units/L.
Renal biopsy
Focal necrotizing glomerulonephritis.
Treatment:
High dose steroids
Cyclophosphamide
Plasma exchange
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?
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.
what is focal and segmental glmerulonephritis?
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.
what is the treatment for focal and segmental glmerulonephritis?
General measures, as previously described.
Trail of steroids, positive response , even partial remission, carries better prognosis.
Alternative options: cyclosporin, cyclophosphamide, and Rituximab.
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?
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.
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?
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.
