glomerular disease Flashcards
what is glomerulonephritis
inflammation of the glomeruli in the kidneys
what is nephritis
inflammation of the kidneys
5 clinical features of nephritic syndrome
haematuria
proteinuria (,3g, +/++ on urinalysis)
oliguria
hypertension
fluid retention
what is oliguria
reduced urine output
mnemonic to remember the causes of nephritic syndrome
SHARP AIM
SLE
HSP
Anti-GBM disease
RPGN
Post-infectious GN, eg. streptococcal, infective endocarditis
Alport syndrome
IgA nephropathy
MPGN
pathophysiology of nephritic syndrome
damage to the glomerular capillary wall - leading to haematuria and reduced kidney function
pathophysiology of nephrotic syndrome
loss of the glomerular basement membrane’s charge and size selectivity resulting in massive proteinuria
what is nephritic syndrome
signs and symptoms that occur due to renal inflammation (particularly glomerulonephritis)
what is the most common cause of nephrotic syndrome in children
minimal change disease
what is the most common cause of nephrotic syndrome in older patients
membranous nephropathy
classic presentation of a patient with nephrotic syndrome
major proteinuria, hypalbuminaemia, peripheral oedema
clinical sign of major proteinuria
frothy urine
classic oedema seen in nephrotic syndrome
periorbital
conservative management of nephrotic syndrome
fluid restriction and reduced salt intake
medical management of nephrotic syndrome
corticosteroids
+ thiazide for oedema
name some complications of nephrotic syndrome
infection - loss of immunoglobulins in urine
reduced vit D
subclinical hypothyroidism (loss of thyroid binding globulin)
what is rapidly progressive glomerulonephritis
acute and severe development of renal impairment
what is the most common subtype of rapidly progressive glomerulonephritis
pauci-immune complex glomerulonephritis
histology of rapidly progressive glomerulonephritis
glomerular crescents
what are glomerular crescents and why do they occur
proliferation of epithelial cells in bowmans space - due to injury of the glomerular capillary wall causing rupture of the glomerular basement membrane
clinical presentation of rapidly progressive glomerulonephritis
rapid deterioration (duh)
severe nephritic syndrome
name some ANCA negative causes of rapidly progressive glomerulonephritis
goodpastures, HSP, SLE
what is the definitive diagnostic test for rapidly progressive GN
renal biopsy
mainstay of treatment for rapidly progressive GN
immunosuppression
prednisolone or cytotoxics e.g. azathioprine or cyclophosphamide
clinical presentation of minimal change disease
nephrotic syndrome !!!
proteinuria, facial oedema, no real renal impairment
fatigue, weight gain (fluid retention)
what is usually needed for a diagnosis of minimal change disease and what is a positive result
electron microscopy - diffuse podocyte effacement (fused or flattened)
first line management of minimal change disease
prednisolone
what is focal segmental glomerulosclerosis associated with
obesity, HIV, sickle cell, PWID, reflux
clinical presentation of focal segmental glomerulosclerosis
nephrotic syndrome
focal segmental glomerulosclerosis on microscopy
light microscopy: areas of sclerosis
electron microscopy: diffuse podocyte foot process effacement
medical management of focal segmental glomerulosclerosis
prolonged glucocorticoid steroids
what antibody is present in most patients with primary membranous nephropathy
anti-PLA2r
clinical presentation of membranous nephropathy
nephrotic syndrome
light microscopy of membranous nephropathy
thickened basement membrane, spike and dome pattern on a silver stain
electron microscopy of membranous nephropathy
immune complex deposits in the basement membrane
blood marker for membranous nephropathy
anti-phospholipase A2 antibodies
what drug is used to induce remission of membranous nephropathy
rituximab
what is another name for membranoproliferative GM
mesangiocapillary GN
double contour of basement membrane on biopsy
membranoproliferative GN
what are the 2 primary mechanism of membranoproliferative GN
immune complex deposition and activation of complement
complement dysregulation leading to persistent activation of alternate pathway
what is the most common form of GN worldwide
IgA nephropathy
histology of IgA nephropathy
IgA deposits and mesangial proliferation
what is the role of mesangial cells and where are they found in the kidney
centre of the glomerulus and help support the capillaries
who usually presents with IgA nephropathy
patients under 30
what can commonly precede IgA nephropathy
infection e.g. URTI
what is associated with IgA nephropathy
HSP
classic presentation of IgA nephropathy
recurrent haematuria following 12-72hrs of an URTI or GI infection
first line investigation for IgA nephropathy
urinalysis and MC&S
what is MC&S investigation
microscopy, culture and sensitivities
management of IgA nephritis
salt restriction
treat proteinuria with an ACE or ARB
management of HTN
what is another name for alports syndrome
hereditary nephritis
what is alports syndrome
genetic condition causing disorder of type 4 collagen matrix
inheritance in alports syndrome
X-linked
classic presentation of alports
microscopic haematuria + hearing loss in earlt years
what kind of hearing loss is seen in alports syndrome
sensorineural
diagnostic investigation for alports syndrome and positive result
renal biopsy - variable thickness of the glomerular basement membrane
what is HSP
IgA mediated small vessel vasculitis
who usually presents with HSP
children
what commonly precedes HSP
viral URTI
classic presentation of HSP
tetrad of: rash, arthritis, abdo pain, renal impairment
management of HSP
supportive: fluids, analgesia and rest
name 2 ways in which HSP is monitored
urinalysis and BP monitoring
what causes anderson fabrys disease
deficiency of ⍺-galactosidase A
inheritance seen in anderson fabrys disease
X-linked
dermatological symptom associated with anderson fabrys disease
angiokeratomas on groin, buttocks and lower trunk
management of anderson fabrys disease
fabryzyme
linear deposition of IgG in basement membrane
goodpastures disease
