Systemic Diseases Flashcards
what is the most common systemic disease with renal involvement?
diabetes
what class of diseases is myeloma part of?
dysproteinaemias
what is the pathophysiology of myeloma?
overproduction of the plasma cells produced by B cells that then produce antibodies
these then damage the bone marrow
what is myeloma?
a cancer of plasma cells
what effect do the collections of abnormal plasma cells have in myeloma?
impair production of normal blood cells
results in anaemia
what is a paraprotein?
abnormal antibodies
what can cause renal dysfunction in myeloma?
paraproteins
what is typical patient with myeloma?
60 year old male
what are the symptoms of myeloma?
bone pain weakness fatigue weight loss recurrent infections
what are the signs of myeloma?
anaemia
hypercalcaemia
renal failure
lytic bone lesions
what is the classic presentation of myeloma?
back pain and renal failure
what investigation is done for all myeloma patients and what is this looking for?
a skeletal survey
looking for areas of bone damage
how is myeloma diagnosed?
suspicion bloods urine bone marrow biopsy skeletal survey
what blood tests are done for myeloma?
serum protein electrophoresis
serum free light chain measurement
what are you looking for in serum protein electrophoresis for myeloma?
clonal immunoglobulines
what are you looking for in the urine for myeloma?
proteinuria - bence jones protein
what is the general management for renal failure in myeloma?
stop nephrotoxic drugs
manage hypercalcaemia
dialysis
what is the specific management for myeloma?
chemotherapy
stem cell transplant
what is amyloid?
insoluble protein fibrils
what is amyloidosis?
the deposition of extracellular amyloid in tissues
what causes amyloidosis?
abnormal folding of proteins that aggregate and become insoluble
what are the four most common forms of amyloidosis?
AL
AA
AB2M
ATTR
what is AL amyloidosis also called?
primary or light chain amyloidosis
what is AA amyloidosis also called?
secondary/systemic/inflammatory
what is AB2M amyloidosis also called?
dialysis amyloidosis
what is ATTR amyloidosis also called?
hereditary and old age amyloidosis
what are the two types of amyloidosis that are seen?
AA
AL
what causes AL amyloidosis?
production of abnormal IG light chains that enter the blood and cause deposits
what organs are commonly affected by AL amyloidosis?
heart bowel skin nerves kidneys
what is the average age of diagnosis of AL amyloidosis?
55-60 years
what is AA amyloidosis associated with?
systemic inflammation
what causes AA amyloidosis?
production of the acute phase protein serum amyloid A protein
what organs are commonly affected by AA amyloidosis?
liver
spleen
kidneys
adrenals
how does amyloidosis affecting the kidneys present?
proteinuria
impaired renal function
what investigations are done for renal amyloidosis?
urinalysis
renal function bloods
renal biopsy
what staining is used for a renal biopsy in amyloidosis?
congo red staining
goes green under polarised light
what scan can be used to show the extent of amyloidosis?
SAP scan
what is vasculitis?
inflammation of the blood vessels
what are the three types of vasculitis?
large
medium
small vessel
what type of vasculitis commonly affects the kidneys?
small vessel
what investigations are done for vasculitis affecting the kidneys?
urinalysis
bloods
immunology
renal biopsy
what is seen in urinalysis of vasculitis?
haematuria
proteinuria
what antibodies are seen in vasculitis?
ANCA
what are the two types of ANCA?
p ANCA
c ANCA
what is another name for p-ANCA?
anti MPO
what is another name for c-ANCA?
anti PR3
how is vasculitis managed?
immunosuppression
plasma exchange
what is the most frequently observed abnormality in lupus nephritis?
proteinuria
