Kidneys in Systemic Disease Flashcards
What is myeloma? What happens?
Aggressive cancer of plasma cells > B cells that produce antibodies. The abnormal plasma cells accumulate in the bone marrow and impair function of normal blood cells. Many Ig molecules produced by myeloma cells are incompletely formed and unattached light chains can be released.
Describe the structure of an antibody?
Two heavy chains form two sides of Y
Two light chains sit along the top bits of the Y
The top half of heavy chains and light chains forms the variable Fab region
The bottom half of the heavy chains forms the constant Fc region
Describe how myeloma can damage the kidneys?
Due to low molecular weight light chains can pass through glomerular filtrate and cause damage to the epithelial cells as protein precipitates as casts.
The light chains can also pass through capillaries and cause damage to the kidneys and other organs that way.
Myeloma can also cause hypercalcaemia due to metastatic bone destruction which can further damage the kidneys.
Myeloma is a disease of young or old people?
Old people
Peak age= 70
Classic presentation of myeloma?
Back pain and AKI
Punched out skeletal lesions?
Myeloma
Symptoms and signs of myeloma?
Renal failure, Bone pain, weakness, fatigue, weight loss, recurrent infections
Anaemia and hypercalcaemia
Describe investigations for myeloma?
Test for anaemia and hypercalcaemia
Urine for Bence Jones protein
Bloods for serum protein electrophoresis and serum free light chains
Skeletal survey for punched out lesions
Renal biopsy- congo red staining for apple green birenfringence
Bone marrow biopsy
Treatment of myeloma?
Renal treatment is supportive
Haematology for cancer treatment
Bence Jones protein in urine?
Myeloma
What is the most common renal disease associated with myeloma?
Light chain nephropathy
Describe what amyloidosis is?
Disease where you get extracellular amyloid in tissues or organs due to abnormal folding of proteins, these abnormal proteins aggregate and become insoluble
What causes amyloidosis?
Many causes, over 30 different proteins identified and there are inherited and acquired forms
4 most common types of amyloidosis in order from most common?
Primary light chain
AA amyloidosis- due to systemic inflammatory disease e.g. RA or IBD
Dialysis
Hereditary and old age
Renal presentation of amyloidosis?
Usually nephrotic syndrome > proteinuria and impaired function
Cardiac presentation of amyloidosis?
Note not in everyone
Restrictive cardiomyopathy- heart can’t fill aswell, diastolic dysfunction get breathlessness, fatigue elevated JVP with diastolic collapse
Neurological presentation of amyloidosis?
Not in all amyloidosis
Neuropathy
GI presentation of amyloidosis?
Not in all amyloidosis
Hepato and splenomegaly
GI malabsorption