Kidneys in Systemic Diseases Flashcards
what is diabetic nephropathy?
small vessel damage due to poor glycaemic control
presentation of diabetic nephropathy
proteinuria
other small vessel damage
poor control signs/ history
management of diabetic nephropathy
ACEI (watch for dry cough)
good control HbA1c
may need RRT
what is myeloma?
dysproteinaemia that is a cancer of plasma cells (produce antibodies)
what happens in myeloma?
there is abnormal plasma cells accumulating in bone marrow resulting in reduced production of normal blood cells
what antibody produced in myeloma causes renal dysfunction?
paraprotein (Ab)
presentation of myeloma
bone pain (lytic bone lesions, hypercalcaemia) weakness, fatigue (anaemia) weight loss recurrent infections renal failure
classic presentation of myeloma
back pain renal failure (AKI)
renal manifestations in myeloma
- glomerular= AL amyloidosis and Ig deposition
- tubular= light chain cast nephropathy
- miscellaneous= dehydration, hypercalcaemia, contrast, bisphosphonates, NSAIDs (important to look at whole history)
diagnosis of myeloma
bloods for serum protein electrophoresis and serum light chains urine for Bence Jones protein (BJP) bone marrow biopsy skeletal survey renal biopsy
management of myeloma
chemotherapy
stem cell transplant
stop nephrotoxics
manage hypercalcaemia (saline +/- bisphosphonates)
plasma exchange to remove light chains (supportive= dialysis)
what is amyloidosis?
deposition of insoluble, abnormally folded proteins
four types of amyloidosis
- primary/ light chain (AL)
- secondary/ systemic/ inflammatory (AA)
- dialysis
- hereditary and old age
what does AL amyloidosis produce?
abnormal immunoglobulin light chains from plasma cells
age group of AL amyloidosis
55-60
what does AA amyloidosis produce?
acute phase protein (serum amyloid A)
what is AA amyloidosis associated with?
inflammatory conditions such as RA, IBD, psoriasis, TB, osteomyelitis, bronchiectasis
what organs does AA amyloidosis affect?
liver
spleen
kidneys
adrenals
presentation of amyloidosis
nephrotic proteinuria +/- impaired function cardiomyopathy peripheral or autonomic neuropathy hepatomegaly/ splenomegaly GI absorption
diagnosis of amyloidosis
urinalysis and bloods
renal biopsy with Congo Red staining
SAP scan
what is Congo red staining?
apple green birefringence under polarised light
what is an SAP scan?
scintigraphy with radiolabelled serum amyloid
management of AA amyloidosis
treat underlying condition
management of AL amyloidosis
immunosuppression such as:
- steroids
- chemotherapy
- stem cell transplant
define vasculitis
inflammation of blood vessels
what are the three small vessel ANCA vasculitis types
- GPA
- MPA
- eGPA
what are the three small vessel ANCA vasculitis types?
- GPA
- MPA
- eGPA
antibodies in GPA
anti-PR3 Ab
cANCA
what is GPA
necrotising granuloma inflammation
presentation of GPA/what does it affect?
pulmonary/ renal syndrome
antibodies in MPA
anti-MPO Ab
pANCA
what is MPA?
no granulomas
what does MPA affect?
systemic renal lung skin GI nerves
what is eGPA?
vasculitis associated with asthma and eosinophilia
has skin involvement
presentation of vasculitis
fever arthralgia weight loss malaise saddle nose in GPA prodomal symptoms can last weeks-months before specific organ involvement
diagnosis of vasculitis
active urinalysis with protein and blood raised inflammatory markers AKI anaemia renal biopsy has crescent GN and pauci-immune immunology
management of vasculitis
immunosuppression (steroids, cyclophosphamide/ rituximab)
plasma exchange
dialysis
ventilation
what is SLE?
chronic AI inflammatory disease of unknown origin
diagnosis of SLE in terms of lupus nephritis
urinalysis has proteinuria +/- microscopic haematuria
classification of lupus nephritis
ISN classification of I-VI
via renal biopsy
management of lupus nephritis
immunosuppressants such as steroids, azathioprine, cyclophosphamide, etc.
what age is vasculitis most common?
5-7th decade
