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Nephrology chapter of mrcp part 1
Typology: Lecture notes
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Knowing a few key facts is the best way to approach the difficult subject of
glomerulonephritis
Typically presents with nephritic syndrome (haematuria, hypertension)
Rapidly progressive glomerulonephritis - aka crescentic glomerulonephritis
โ rapid onset, often presenting as acute kidney injury โ causes include Goodpasture's, ANCA positive vasculitis
Mixed nephritic/nephrotic presentation
Diffuse proliferative glomerulonephritis
โ classical post-streptococcal glomerulonephritis in child โ presents as nephritic syndrome / acute kidney injury โ most common form of renal disease in SLE
Membranoproliferative glomerulonephritis (mesangiocapillary)
โ type 1: cryoglobulinaemia, hepatitis C
not free, thyroxine levels.
โ minimal change glomerulonephritis (causes 80% in children, 30% in adults) โ membranous glomerulonephritis โ focal segmental glomerulosclerosis โ membranoproliferative glomerulonephritis
โ diabetes mellitus โ systemic lupus erythematosus โ amyloidosis
โ gold (sodium aurothiomalate), penicillamine
โ congenital โ neoplasia: carcinoma, lymphoma, leukaemia, myeloma โ infection: bacterial endocarditis, hepatitis B, malaria
โ increased risk of thromboembolism related to loss of antithrombin III and plasminogen in the urine o deep vein thrombosis, pulmonary embolism o renal vein thrombosis, resulting in a sudden deterioration in renal function โ hyperlipidaemia o increasing risk of acute coronary syndrome, stroke etc โ chronic kidney disease โ increased risk of infection due to urinary immunoglobulin loss โ hypocalcaemia (vitamin D and binding protein lost in urine) โ Nephrotic syndrome predisposes to thrombotic episodes, possibly due to loss of antithrombin III. These commonly occur in the renal veins and may be bilateral. Membranous glomerulonephritis is the most common glomerulonephropathy linked to renal vein thrombosis.
Membranous glomerulonephritis is the commonest type of glomerulonephritis in adults and is the third most common cause of end-stage renal failure (ESRF). It usually presents with nephrotic syndrome or proteinuria.
include female sex, young age at presentation and asymptomatic proteinuria of a modest degree at the time of presentation.
Minimal change disease nearly always presents as nephrotic syndrome, accounting for 75% of cases in children and 25% in adults.
The majority of cases are idiopathic, but in around 10-20% a cause is found:
โ drugs: NSAIDs, rifampicin โ Hodgkin's lymphoma, thymoma โ infectious mononucleosis
Pathophysiology
โ T-cell and cytokine-mediated damage to the glomerular basement membrane โ polyanion loss โ the resultant reduction of electrostatic charge โ increased glomerular permeability to serum albumin
Features
โ nephrotic syndrome โ normotension - hypertension is rare โ highly selective proteinuria o only intermediate-sized proteins such as albumin and transferrin leak through the glomerulus โ renal biopsy o normal glomeruli on light microscopy o electron microscopy shows fusion of podocytes and effacement of foot processes
Management
โ majority of cases (80%) are steroid-responsive โ cyclophosphamide is the next step for steroid-resistant cases
Prognosis is overall good, although relapse is common. Roughly:
โ 1/3 have just one episode โ 1/3 have infrequent relapses โ 1/3 have frequent relapses which stop before adulthood
Focal segmental glomerulosclerosis (FSGS) is a cause of nephrotic syndrome and chronic kidney disease. It generally presents in young adults.
ยฉ Image used on license from PathoPic Sclerosis of the glomerulus is seen next to Bowman's capsule
ยฉ Image used on license from PathoPic Sclerosis is seen in the perihilar region of the glomerulus
The lack of blood in the urine indicates this to be a purely nephrotic syndrome. Given the patient's age, the most likely cause of nephrotic syndrome is focal segmental glomerulosclerosis or membranous glomerulopathy, accounting for approximately a third of cases each.
Diabetic nephropathy may be classified as occurring in five stages*
โ hyperfiltration: increase in GFR โ may be reversible
โ most patients do not develop microalbuminuria for 10 years โ GFR remains elevated
โ microalbuminuria (albumin excretion of 30 - 300 mg/day, dipstick negative)
โ persistent proteinuria (albumin excretion > 300 mg/day, dipstick positive) โ hypertension is present in most patients โ histology shows diffuse glomerulosclerosis and focal glomerulosclerosis (Kimmelstiel-Wilson nodules)
โ end-stage renal disease, GFR typically < 10ml/min โ renal replacement therapy needed