Kidney Disease in Systemic diseases Flashcards
What is dysproteinameia?
over production of immunoglobulin by clonal expansion of cells from B cell lineage
What is a myeloma?
cancer of plasma cells - collections of abnormal plasma cells accumulate in the bone marrow due to dysregulatd proliferation causing impaired production of normal blood cells and monoclonal production of paraprotein which can cause renal dysfunction
What is the characteristic symptoms of myeloma?
back pain and renal failure bone pain weakness fatigue weight loss recurrent infections
What are the signs of myeloma?
anaemia
hypercalcaemia
renal failure
lytic bone lesions
What are the renal manifestations of myeloma?
glomerular = AL amyloidosis, monoclonal immuglobulin deposition tubular = light chain cast nephropathy miscallaneous = dehydration, hypercalcaemiam, contrast, biphosphonates, NSAIDs
What do the kidneys filtering abnormal proteins show?
problems in the glomerulus
How is myeloma diagnosed?
bloods - serum protein electrophoresis, serum free light chains urine - bence jones proteins bone marrow biopsy skeletal survey renal biopsy
What is the management of myeloma?
stop nephrotoxins
manage hypercalcaemia - saline, maybe biphosphonates
chemo
stem cell transplant
What is amyloidosis?
deposition of extracellular amyloid - insoluble protein fibrils - in tissues or organs
What causes amyloidosis?
abnormal folding of proteins which then aggregate together and block normal tissue causing dysfunction
What are the 4 types of amyloidosis?
AL - primary/light chain AA - secondary/systemic/inflammatory 2 minor ones: dialysis hereditory and old age
What is AL amyloidosis?
production of abnormal immunoglobulin light chains from plasma cells
light chains enter the blood stream and cause amyloid deposits
What does AL amyloidosis affect?
heart bowels skin nerves kidneys
What age is affected by AL amyloidosis?
55-60 years
What is the prognosis for AL amyloidosis?
6months - 4 years if untreated
Describe AA amyloidosis?
associated with systemic inflammation
production of the acute phase protein - serum amyloid A
What puts you at a higher risk of AA amyloidosis?
chronic inflammatory conditions or infections
What does AA amyloidosis affect?
liver
spleen
kidneys
adrenals
What is the presentation of amyloidosis?
renal - nephrotic (proteinuria and impaired renal function)
cardiac - cardiomyopathy
nerves - peripheral or autonomic neuropathy
hepato/splenomegaly
GI malabsorption
What investigations can be done for amyloidosis?
urinalysis and uPCR
renal biopsy shows congo red staining - apple green under polarised light
bloods
What is the management of AL amyloidosis?
immunosuppression - steroids, chemo, stem cell transplant
What is the management of AA amyloidosis?
treat underlying condition
What is lupus nephritis?
50% of lupus patients will have renal involemetn at presentation
normally - proteinuria
What should all patients with lupus nephritis be on?
hydroxychloroquine
