the kidneys in systemic disease Flashcards
what is myeloma?
cancer of plasma cells, collections of abnormal plasma cells can accumulate in the bone marrow and/or soft tissue
what are the symptoms of myeloma?
Bone Pain
Weakness
Fatigue
Weight Loss
Recurrent infections
the classic presentation is back pain and renal failure
what are the clinical signs of myeloma?
Anaemia
Hypercalcaemia
Renal Failure
Lytic bone lesions
how do you diagnose myeloma?
rare so high clinical suspicion
bloods- serum protein electrophoresis, free light chains
urine- bence jones protein
bone marrow biopsy
skeletal survey
renal biopsy
what is the management of myeloma?
stop nephrotoxics and manage hypercalcaemia
disease specific- chemotherapy & stem cell transplant
dialysis
what is amyloidosis?
deposition of insoluble extracellular amyloid in tissues or organs due to abnormal protein folding
what are the 4 common forms of amyloidosis?
primary / light chain (AL)
secondary / systemic / inflammatory (AA)
dialysis (AB2M)
hereditary and old age (ATTR)
what is primary amyloidosis (AL)?
production of abnormal immunoglobulin from light chains in plasma cells- light chains enter bloodstream and cause amyloid deposits
commonly affects heart, bowel, skin, nerves and kidneys
life expectancy 6 months-4 years without treatment
what is secondary amyloidosis (AA)?
Production of acute phase protein – serum amyloid A protein (SAA) associated with systemic inflammation e.g. RA, IBD, psoriasis or infection e.g. TB, osteomyelitis, bronchiectasis
commonly affects liver, spleen, kidneys, adrenals, rarer than primary
what is the presentation of amyloidosis?
Renal – (nephrotic range) proteinuria
+/- impaired renal function
Cardiac – Cardiomyopathy
Nerves – peripheral or autonomic neuropathy
Hepatomegaly / Splenomegaly
GI - malabsorption
what are the renal investigations for amyloidosis?
Urinanlysis + uPCR
Blood tests – renal function, markers of inflammation, protein electrophoresis, SFLC
Renal Biopsy
Congo red staining (apple green under polarised light)
what is the management of amyloidosis?
not curative- preserves organ function
AA- treat underlying cause
AL- immunosuppression - steroids, chemotherapy, stem cell transplant
refer to national amyloidosis centre
how does small vessel vasculitis work?
Necrotising polyangiitis that affects capillaries, venules and arterioles
how does small vessel vasculitis work?
Necrotising polyangiitis that affects capillaries, venules and arterioles
how do you diagnose vasculitis?
High index of suspicion
Urinalysis – “active urine” blood++ protein++
Bloods (raised inflammatory markers, AKI, anaemia)
Immunology
Anti-Neutrophil cytoplasmic antibody (ANCA)
Anti – MPO - pANCA
Anti – PR3 - cANCA
Renal Biopsy – crescentic GN, pauci-immune
