kidneys in systemic disease Flashcards
what is myeloma?
a cancer of plasma cells ( a cell that is usually responsible for producing antibodies)
results in abnormal antibody production
who is usually affected by myeloma?
men about 60 years old
which protein is found in the urine in myeloma?
bence jones protein
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?
middle aged
back/bone pain
renal failure
what are the glomerular renal manifestations of myeloma?
AL amyloidosis
monoclonal immunoglobulin deposition (light/heavy chains)
what are the tubular renal manifestations of myeloma?
light chain cast nephropathy
how is myeloma diagnosed?
bloods
-serum protein electrophoresis (clonal Ig)
- serum free light chains (kappa/lambda)
urine
-bence jones protein
confirmed with a bone marrow biopsy
how is myeloma managed?
general measures (stop nephrotoxics and manage hypercalcaemia)
chemo and stem cell transplant
dialysis?
what is amyloidosis?
the deposition of extracellular amyloid (insoluble protein fibrils) in tissues or organs
what are the 4 “common” forms of amyloidosis?
primary/light chain (AL)
secondary/systemic/inflammatory (AA)
dialysis (AB2M)
hereditary and old age (ATTR)
how does primary/light chain amyloidosis occur?
through the production of abnormal immunoglobulin light chains from plasma cells
light chains enter the blood stream and cause amyloid deposits
what does primary amyloidosis commonly affect?
heart
bowel
skin
nerves
kidneys
what age group is usually affected by primary amyloidosis?
55-60 year olds
life expectancy after diagnosis 6mths-4years (untreated)
what causes secondary amyloidosis?
production of acute phase protein SAA
develops in approx 5% of patients with chronic inflammatory conditions/infections
what is the presentation of amyloidosis?
depends on organs involved
renal
- proteinuria +/- impaired renal function
cardiac
-cardiomyopathy
nerves
-peripheral or autonomic neuropathy
hepatomegaly/splenomegaly
how is amyloidosis investigated?
urinalysis
bloods
renal biopsy
SAP scan
what is the management of amyloidosis?
non curative
aim to reduce more deposition and preserve organ function
AA-treat underlying condition
AL-immunosuppression (steroids, chemo, stem cell transplant)
what is small vessel vasculitis?
a necrotising polyangiitis
usually presents in 5th, 6th, 7th decade
presentation is very vague
how is vasculitis diagnosed?
urinalysis
- blood and protein
immunology
-anti MPO (pANCA)
- anti PR3 (cANCA)
what percentage of EGPA is pANCA associated?
40%
what is GPA associated with?
lung involvement
saddle nose
what is EGPA associated with?
asthma and eosinophilia
2/3 have skin involvement
how is ANCA vasculitis treated?
immunosuppression
-steroids
-cyclophosphamide/rituximab
plasma exchange
supportive
how is SLE disgnosed?
young lady with vague symptoms
bloods
- raised inflammatory markers
-+ve ANA, anti dsDNA
- low levels of complement (C3 and C4)
urinalysis
- proteinuria+/- microscopic haematuria (very common in renal involvement)
