systemic disease and the kidneys Flashcards
why can myeloma cause renal dysfunction
abnormal plasma cells produce excessive amounts of antibodies, which can obstruct filtration in the kidneys
classic presentation of myeloma
CRAB
calcium elevated
renal failure
anaemia
bone lesions and bone pain - BACK!!
name some investigations done in suspected myeloma and a positive result
serum protein electrophoresis - paraprotein spike
serum bence jones protein !!
what is the most common complication of myeloma
anaemia
what investigation is used to confirm the diagnosis of myeloma
bone marrow biopsy
pepper pot skull on xray might indicate…
multiple lytic lesions in the skull
may indicate myeloma
pathophysiology of amyloidosis
depositions of extracellular fibrils (misfolded proteins) in organs and blood vessels - causing dysfunction
what are the 2 most common types of amyloidosis
primary - AL amyloidosis
secondary - AA amyloidosis
what causes AL amyloidosis
deposition of monoclonal light chains from abnormal plasma cells
what condition can commonly cause AL amyloidosis
myeloma
what causes AA amyloidosis
systemic chronic inflammation causing the production of amyloid A protein
what is a common condition that can lead to AA amyloidosis
rheumatoid arthritis
what can periorbital purpura, ‘racoon eyes’ be a sign of
amyloidosis
classic renal presentation of amyloidosis
nephrotic syndrome
diagnostic investigation for amyloidosis
tissue biopsy shows apple green bifringence when stained with congo red under polarised light
what investigation can be used to determine the extent of disease in amyloidosis
SAP scans
management of AL amyloidosis
immunosuppression
what is small vessel vasculitis
inflammation of the small blood vessels, including capillaries, venules and arterioles
what are the 3 main ANCA-associated vasculitis
granulomatosis with polyangiitis
microscopic polyangiitis
eosinophilic granulomatosis with polyangiitis
what is the old name for granulomatosis with polyangiitis
wegener’s granulomatosis
what is commonly affected in granulomatosis with polyangiitis
respiratory tract and the kidneys
classic URT sign of granulomatosis with polyangiitis
saddle shaped nose due to nasal bridge collapse
presentation of granulomatosis with polyangiitis
URT: sinusitis, nose bleeds, hearing problems
LRT: cough, wheeze, haemoptysis
renal: rapidly progressing glomerulonephritis
antibodies associated granulomatosis with polyangiitis
anti-PR3 (cANCA)
antibodies associated with microscopic polyangiitis
anti-MPO (pANCA)
classic presentation of microscopic polyangiitis
glomerulonephritis and haemoptysis
what causes haemoptysis in microscopic polyangiitis
diffuse alveolar haemorrhage
what is another name for eosinophilic granulomatosis with polyangiitis
churg strauss
antibody associated with eosinophilic granulomatosis with polyangiitis
anti-MPO (pANCA)
classic presentation of eosinophilic granulomatosis with polyangiitis
severe asthma in middle age and raised eosinophils on FBC
small vessel vasculitis on renal biopsy
crescentic GN, pauci-immune
management of small vessel vasculitis
immunosuppression
what is systemic lupus erythematous (SLE)
inflammatory autoimmune conntective tissue disorder
what is the usual clinical course of SLE
relapsing-remitting, with intermittent flares of disease
most frequent renal abnormality in SLE
proteinuria
autoantibodies in a patient with SLE
ANA, anti-dsDNA and anti-sm
key diagnostic test for lupus nephritis
renal biopsy
fist line management of SLE
hydroxychloroquine
what is the leading cause of kidney failure in the UK
diabetic nephropathy
what is diabetic nephropathy
kidney damage due to long-standing hyperglycaemia in the context of diabetes
Kimmelstiel-Wilson lesions
diabetic nephropathy!!
what is diabetic nephropathy commonly associated with
retinopathy
how do we define diabetic nephropathy
albuminuria and progressive reduction in eGFR in the context of long duration of diabetes
main investigation for diabetic nephropathy
biopsy: thickening of the glomerular basement membrane
first line management of diabetic nephropathy
ACEi
second line management of diabetic nephropathy
SGLT2i
what is another name for goodpasture syndrome
anti-GBM
what causes goodpasture syndrome
antibodies (IgG) against a3 subunit of type 4 collagen
what kind of hypersensitivity reaction is goodpastures syndrome
type 2
what are the 2 main clinical presentations of goodpastures
rapidly progressive glomerulonephritis and pulmonary haemorrhage
management of goodpastures
plasmapheresis
gold standard diagnosis of goodpastures and a positive result
renal biopsy
cellular crescents and focal necrosis, linear IgG deposition
