Glomerular disease, clinical

Question 1

What is the nephritic state?

Answer 1

• Active urine sediment: haematuria, dysmorphic RBCs, cellular casts
• Hypertension
• Renal impairment

Question 2

What is nephrotic syndrome?

Answer 2

• Oedema
• Proteinuria >3.5 g/day
• Hypoalbuminemia
• Hyperlipidemia
• Can be caused by primary (idiopathic) glomerular disease or secondary glomerular diseases

Question 3

What is proliferative glomerulonephritis?

Answer 3

Excessive numbers of cells in glomeruli. These include infiltrating leucocytes

Question 4

What is non-proliferative glomerulonephritis?

Answer 4

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

Question 5

What is post streptococcal glomerulonephritis?

Answer 5

• Follows 10-21 days after infection typically of throat or skin
• Most commonly with Lancefield group A Streptococci.
• Genetic predisposition: HLA-DR, -DP, -DP.

Question 6

What is the treatment of streptococcal glomerulonephritis?

Answer 6

• Antibiotics for infection, debatable.
• Loop diuretics such as furosemide for oedema
• Vasodilator drugs (e.g. amlodipine) for hypertension

Question 7

What is IgA nephropathy?

Answer 7

• Characterized by IgA deposition in the mesangium +mesangial proliferation.
• Most common in 2nd and 3rd decade of life with males more commonly affected.
• Up to 40% of cases can progress to end stage kidney disease.

Question 8

What is the presentation of IgA nephropathy?

Answer 8

• Microscopic haematuria.
• Microscopic haematuria + proteinuria
• Nephrotic syndrome
• IgA crescentic glomerulonephritis

Question 9

What is the management of crescentic glomerulonephritis?

Answer 9

Immunosuppression

• Corticosteroids
• Plasma exchange
• Cytotoxic e.g. Cyclophosphamide
• B-cell therapy e.g. Rituximab
• Complement inhibitors

Question 10

What is minimal change nephrotic syndrome?

Answer 10

• Commonest form in children
• Sudden onset of oedema: days
• Complete loss of proteinuria with steroids
• Two thirds of patients relapse

Question 11

What is the treatment for minimal change disease?

Answer 11

• Prednisolone – 1mg/kg for up to 16 weeks.
• Once remission achieved , slow taper over 6 months.
• Initial relapse treated with further steroid course.
• Subsequent relapses treated with
• Cyclophosphamide
• Cyclosporin
• Tacrolimus
• Mycophenolate mofetil
• Rituximab

Question 12

What is focal and segmental glomerulonephritis?

Answer 12

• 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 13

What is the treatment for focal and segmental glomerulonephritis?

Answer 13

• General measures
• Trial of steroids, positive response, even partial remission, carries better prognosis
• Alternative options: cyclosporine, cyclophosphamide, and Rituximab

Question 14

What is membranous nephropathy?

Answer 14

• Commonest cause of nephrotic syndrome in adults
• Majority of cases occur in isolation (idiopathic).
• Serological markers
• Anti-phospholipase A2 receptor (PLA2R) antibody positive in 70% of idiopathic cases
• Thrombospondin type 1 domain containing 7A (THSD7A).
• Secondary causes include:
• Malignancies
• SLE
• Rheumatoid arthritis.
• Drugs: NSAIDs, gold, penicillamine.

Question 15

What is the treatment for membranous nephropathy?

Answer 15

• General measures for at least 6 months.
• Immunosuppression if symptomatic nephrotic syndrome, rising proteinuria or deteriorating renal function.
• Cyclophosphamide and steroids (alternate months) for 6 months.
• Cyclosporine.
• Rituximab.