Glomerulonephritis with nephritic and nephrotic syndrome

Question 1

1) What is nephritis?
2) What is nephritic syndrome?
3) How does nephrotic and nephritic syndrome differ in terms of what they represent?
4) Name 2 features of nephritic syndrome
5) Name 3 of the features a patient must have to have nephrotic syndrome

Answer 1

1) General term of inflammation within the kidneys
2) Nephritic syndrome = group of symptoms and NOT a diagnosis. It means that patients fit a clinical picture of having inflammation of their kidney
3) There are no set criteria for having nephritic syndrome, whereas there is for nephrotic syndrome
4) Haematuria, oliguria, proteinuria, fluid retention
5) Proteinuria, oedema, hypoalbuminemia, hypercholesterolaemia

Question 2

1) What is glomerulonephritis?
2) What is glomerulosclerosis?
3) Name 2 things that can cause glomerulosclerosis

Answer 2

1) Umbrella term applied to conditions that cause inflammation of or around the glomerulus and nephron
2) Term to describe the pathological process of scarring of the tissue in the glomerulus
3) Any cause of glomerulosclerosis, focal segmental glomerulosclerosis, obstructive neuropathy

Question 3

Nephrotic glomerulonephritis (1)
1) Name 2 things nephrotic glomerulonephropathies predispose patients to
2) Why are ACEi/ARBs used in the management of most nephrotic glomerulosclerosis (2)?
3) What is the commonest cause of nephrotic syndrome in children?
4) What is normally the cause of this condition?
5) Name another cause
6) What can be seen on electron microscope in this condition?
7) What is it normally treated with?

Answer 3

1) Hypertension, hypercholesterolemia, thrombosis, infection
2) Reduce proteinuria + BP control
3) Minimal change disease
4) Idiopathic
5) Drugs i.e. NSAIDs, rifampicin or Hodgkin’s lymphoma
6) Fusion of podocytes and effacement of foot processes
7) Prednisolone (oral corticosteroids). Cyclophosphamide = second line

Question 4

Nephrotic glomerulonephropathies (2)
1) What is the commonest cause of nephrotic syndrome in adults?
2) Name 2 causes of this condition
3) This condition is known to have a high recurrence rate following what?
4) How is this condition investigated/diagnosed?
5) What group of drugs is 1st line?

Answer 4

1) Focal segmental glomerulosclerosis
2) Idiopathic, secondary to other renal pathology i.e. IgA nephropathy, reflux nephropathy, HIV, heroin, Alport’s syndrome, sickle-cell disease
3) Renal transplant
4) Renal biopsy
5) Steroids

Question 5

Nephrotic glomerulonephropathies (3)
1) What is the commonest type of glomerulonephropathy overall?
2) Name a primary cause
3) Name a secondary cause
4) What is seen on histology in this condition?
5) What is the cause in the majority of idiopathic cases?
6) How is it managed in patients with high risk of progression?

Answer 5

1) Membranous glomerulonephritis
2) Idiopathic
3) Malignancies i.e. prostate, lung, lymphoma, leukaemia, infections i.e. malaria, hepB, drugs i.e. gold, penicillamine, NSAIDs. SLE, rheumatoid arthritis
4) IgG and complement deposits on the basement membrane
5) Phospholipase A2 autoantibody
6) Corticosteroid + other immunosuppressive drug i.e. cyclophosphamide

Question 6

Nephrotic glomerulonephropathies (4)
1) Membranoproliferative/mesangiocapillary glomerulonephritis can be divided into 2 types - what are main way that they’re differentiated?
2) Which is more common?

Answer 6

1) Type 1 = immune complex associated. Type 2 = C3 glomerulopathy
2) Type 1 (90%)

Question 7

Nephritic glomerulonephropathies (1)
1) What is the commonest cause of primary glomerulonephritis?
2) How does it normally present?
3) What does histology show?
4) What is Henoch-Schonlein purpura?
5) Name 2 ways it can present

Answer 7

1) IgA nephropathy
2) Macroscopic haematuria followed by an upper respiratory tract infection
3) IgA deposits and glomerular mesangial proliferation
4) Small vessel vasculitis and a systemic variant of IgA nephropathy
5) Polyarthritis, abdo pain, purpuric rash over buttock and extensor surface of arms and legs, IgA nephropathy features

Question 8

Nephritic glomerulonephropathies (2)
1) When does post-streptococcal glomerulonephritis (PSGN) typically occur?
2) Name 2 ways it can present
3) Name 2 other things/results of investigations that points towards a diagnosis of PSGN
4) What happens in anti-GBM disease?
5) What 2 things can this cause?
6) How can these 2 things present?
7) What are the 3 aspects of the management of anti-GBM disease?

Answer 8

1) 7-14 days following a group A beta-haemolytic Streptococcus infection (skin or sore throat)
2) Haematuria, proteinuria (results in oedema), oliguria, htn
3) Low C3/C4, red cell casts in urine, +ve antistreptolysin O titre, positive anti-DNase antibody
4) Anti-GBM antibodies attack the glomerulus and pulmonary basement membranes
5) Glomerulonephritis and pulmonary haemorrhage
6) Haemoptysis, haematuria
7) Plasma exchange, steroids, cyclophosphamide

Question 9

Nephritic glomerulonephropathies (3)
1) What is rapidly progressive glomerulonephritis?
2) Name a cause
3) How is it treated (3)?

Answer 9

1) Rapid loss of renal function associated with the formation of epithelial crescents in the majority of glomeruli
2) Anti-GBM disease, SLE
3) Corticosteroid + cyclophosphamide + plasma exchange