Kidneys in systemic disease Flashcards
what is myeloma
cancer of plasma B cells which causes dysproteinaemias
what does myeloma dysproteinaemia cause
overproduction of immunoglobulin proteins by expansion of B cell lineage and monoclonal production of paraprotein
what is the classic presentation of myeloma
back pain, bone pain and AKI/ renal failure
what are symptoms of myeloma (5)
bone pain, weakness, fatigue, weight loss, recurrent infections
what are signs of myeloma (4)
anaemia, hypercalcaemia, renal failure, lytic bone lesions
what renal manifestations of myeloma are there
build up of proteins, glomerular: AL amyloidosis, immunoglobulin deposits // tubular: light chain cast nephropathy
how do you diagnose myeloma
bloods: FBC + electrophoresis - free light chains // urine: bence Jones proteins // CT: lytic bone legions // bone marrow and renal biopsy
how do you manage myeloma
saline +/- biphosphonates (hypercalcaemia) // dexamethasone, cyclophosphamide (chem), stem cell transplant, dialysis
what is amyloidosis
deposition of extracellular amyloid (insoluble proteins) in tissues and organs
what are the 2 most common forms of amyloidosis
primary light chains (AL) // secondary, systemic, inflammatory (AA) - both commonly linked to myeloma
what abnormal protein is produced in AL amyloidosis
abnormal immunoglobulin light chains from plasma cells
where does AL amyloidosis commonly cause deposits (5)
heart, bowel, skin, nerves, kidneys
what age does AL amyloidosis commonly present in and what is the life expectancy
55-60, <4 years if untreated
how do you treat AL amyloidosis
immunosuppression: steroids, chemo, stem cell transplant
what abnormal protein is produced in AA amyloidosis
acute phase protein (SAA)
who commonly develops AA amyloidosis
patients with chronic inflam conditions or chronic infections
where does AA amyloidosis commonly cause deposits (4)
liver, spleen, kidneys, adrenals
what are symptoms of amyoidosis (where it affects)
nephrotic syndrome!!! proteinuria +/- renal function, cardiomyopathy, neuropathy: (numbness, tingling), hepatosplenomegaly, GI malabsorption: (nausea, diarrhoea)
how do you diagnose amyloidosis
renal biopsy: congo reed staining (apple green bifrigence)
what other investigations can be done for amyloidosis
urinalysis, blood tests (RFT, inflam), SAP scan - extent of disease
what types of vasculitis commonly affect the kidneys
small vessel ANCA positive: GPA and MPA
how do you diagnose vasculitis
urinalysis, renal biopsy, blood antibodies: anti PR3 cANCA for GPA /// anti MPO pANCA for MPA
how do you manage vasculitis
immunosuppression: steroids, cyclophosphamide, plasma exchanged, dialysis
what is the most common presentation in SLE affecting the kidneys
proteinuria
what is the most common cause of renal failure
T2DM
which arteriole is affected more in diabetes and what does this result in
efferent > afferent, results in initial increased GFR which causes hyperfiltration and microalbinuria
how does T2DM cause renal failure
glycolysation causes leaky capillaries –> hyperfiltration in the early stages –> type IV collagen deposits
how do you treat T2DM kindey failure
control BP with ACEi and ARBs, insulin control, statins, dialysis (bad prognosis)
