Glomerulonephritis and Nephrotic Syndrome Flashcards
What is glomerulonephritis?
Group of inflammatory disorders, mostly due to autoimmunity, that destroy the glomeruli of the nephron and are classified by morphology/shape
What are the types of glomerulonephritis?
Non-proliferative, generally causes nephrotic
- Minimal change disease
- Focal Segmental Glomerulosclerosis
- Membranous glomerulonephritis
Proliferative, generallt causes nephritis
- Post-streptococcal
- IgA nephropathy
- Membranoproliferative glomerulonephritis
What is the most common glomerulonephritis in children?
Minimal change disease
What is the most common glomerulonephritis in adults?
IgA nephropathy
What is IgA nephropathy?
Also known as Berger’s disease, autoimmune glomerulonephritis classically presenting as macroscopic haematuria in young people following an upper respiratory tract infection
What conditions is IgA nephropathy associated with?
Alcoholic cirrhosis
Coeliac disease/dermatitis herpetiformis
Henoch-Schonlein purpura
How does IgA nephropathy present?
Young male
Recurrent episodes of macroscopic haematuria
Typically associated with a recent respiratory tract infection, developing 1-2 days after
How is IgA nephropathy managed?
BP control, as steroids/immunosuppresants not shown to be effective
What causes membranous glomerulonephritis?
Idiopathic
Infections
- Hepatitis B
- Malaria
- Syphilis
Malignancy
- Prostate, lung, lymphoma, leukaemia
Drugs
- Gold
- Penicillamine
- NSAIDs
Autoimmune diseases
- SLE
- Thyroiditis
- RA
How does membranous glomerulonephritis present?
Nephrotic syndrome
Proteinuria
How is membranous glomerulonephritis managed?
ACEI/ARB
Immunosuppression in severe or progressive disease
Combination of corticosteroid + another agent such as cyclophosphamide
Anticoagulation for high-risk patients
What biopsy sign is seen in membranous glomerulonephritis?
Basement membrane is thickened with subepithelial electron dense deposits, creating a ‘spike and dome’ appearance
What antibody is associated with membranous glomerulonephritis?
Anti-Phospholipase A2 Receptor (PLA2R)
How does post-streptococcal glomerulonephritis present?
Typically occurs in children 7-14 days post streptococcal infection
Headache
Malaise
Visible haematuria
Proteinuria
HTN
Oliguria
How is post-streprotoccal glomerulonephritis managed?
Antibiotics
What causes minimal change disease?
Idiopathic
Drugs
- NSAIDs
- Rifampicin
Hodgkin’s lymphoma
Thymoma
Infectious mononucleosis
How does minimal change disease present?
Nephrotic syndrome
Normotension
Highly selective proteinuria, only intermediate size proteins leak through
How is minimal change disease managed?
Prednisolone for 16 weeks and slow taper over 6 weeks
Further steroids for initial relapse
Cyclophosphamide for steroid resistant cases
What biopsy sign is seen in minimal change disease/non-proliferative glomerulonephritis?
Fused podocytes
What is the prognosis of minimal change disease?
1/3 have just one episode
1/3 have infrequent relapses
1/3 have frequent relapses which stop before adulthood
What causes focal segmental glomerulosclerosis?
Idiopathic
Secondary to other renal pathology
- IgA nephropathy
- Reflux nephropathy
HIV
Heroin
Alport’s syndrome
Sickle-cell
How is focal segmental glomerulosclerosis managed?
Immunosuppression
What are the two types of membranoproliferative glomerulonephritis?
Anti Neutrophil Cytoplasmic Antibody (ANCA)
Anti-Glomerular Basement Membrane (GBM)
How is membranoproliferative glomerulonephritis managed?
High dose steroids
Cyclophosphamide/Immunosuppression
Plasma exchange
What is rapidly progressive glomerulonephritis?
Describes the rapid loss of renal function associated with the formation of epithelial crescents in the majority of glomeruli, presenting as nephritis syndrome
What is nephrotic syndrome?
Damage to the glomerular basement membrane resulting in a triad of proteinuria (> 3g/24hr) causing hypoalbuminaemia (< 30g/L) and oedema
What is Goodpastures syndrome?
Rare small-vessel vasculitis associated with both pulmonary haemorrhage and rapidly progressive glomerulonephritis, caused by anti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen
What group is Goodpasture’s syndrome more common in?
Young males
How does Goodpasture’s present?
Pulmonary haemorrhage
Rapidly progressive glomerulonephritis, typically resulting in a rapid onset acute kidney injury
- Proteinuria + haematuria
What investigations are used in Goodpasture’s diagnosis?
Renal biopsy
- Linear IgG deposits along the basement membrane
Raised transfer factor secondary to pulmonary haemorrhages
How is Goodpasture’s managed?
Plasmapheresis
Steroids
Cyclophosphamide
How is nephrotic syndrome managed?
Oedema: Loop diuretics, Salt/fluid restriction
HTN: RASS blockage
Thrombosis Risk: Heparin, Warfarin
Hyperlipidaemia: Statins
Give complications of nephrotic syndrome
DVT
PE
Renal vein thrombosis, resulting in a sudden deterioration in renal function
Hyperlipidaemia
Acute coronary syndrome, stroke etc
chronic kidney disease
Infection
Hypocalcaemia
What is haemolytic uraemic syndrome?
Combination of haemolysis with red cell fragmentation, thrombocytopenia and AKI
What causes rapidly progressive glomerulonephritis?
Goodpasture’s syndrome
Wegener’s granulomatosis
SLE
Microscopic polyarteritis
