kidneys in systemic disease Flashcards
systemic causes of CKD
- myeloma
- amyloidosis
- vasculitis
- SLE
what is myeloma
cancer of plasma cells
- collection of abnormal plasma cells can accumulate in the bone marrow and/or soft tissue
- impairment of production of normal blood cells > anaemia
- monoclonal production of a paraprotein could cause renal dysfunction
presentation of myeloma
- bone pain
- weakness
- fatigue
- weight loss
- recurrent infections
signs of myeloma
- anaemia
- hypercalcaemia
- renal failure
- lytic bone lesions
renal manifestations of myeloma
Glomerular
- AL amyloidosis
- monoclonal immunoglobulin deposition
Tubular
-light chain cast nephropathy
-dehydration/hypercalcaemia,
diagnosis of myeloma
bloods (proteins, free light chains) urine (bence jones protein) bone marrow biopsy skeletal survey renal biopsy
usually protein electrophoresis and BJP
management of myeloma
- stop nephrotoxics
- manage hypercalcaemia
- chemotherapy
- stem cell transplant
- dialysis
how do you manage hypercalcaemia
saline +/- bisphosphonates
what is amyloidosis
deposition of extracellular amyloid in tissues or organs
-occurs due to abnormal folding of proteins which then aggregate and become insoluble
what are the four forms of amyloidosis
- primary/light chain (AL)
- secondary/systemic/inflammatory (AA)
- dialysis
- hereditary and old age
what is AL amyloidosis
- production of abnormal immunoglobulin light chains from plasma cells
- light chains enter bloodstream and cause amyloid deposits
what does AL amyloidosis commonly affect
- heart
- bowel
- skin
- nerves
- kidneys
who usually gets AL amyloidosis
55-60yrs
what is AA amyloidosis
- associated with systemic inflammation
- production of acute phase protein - serum amyloid A protein
what does AA amyloidosis affect
- liver
- spleen
- kidneys
- adrenals
presentation of amyloidosis
depends on organ or tissues involved
- renal - proteinuria +/- impaired renal function
- cardiomyopathy
- peripheral or autonomic neuropathy
- hepatomegaly/splenomegaly
- malabsorption
renal investigation for amyloidosis
-urinalysis + uPCR
-blood tests
-renal biopsy (congo red staining)
-
management for amyloidosis
no cure
- preserve organ function and reduce further deposition
- AA - treat underlying cause
- AL - immunosuppression - steroids, chemo, stem cell transplant
what is ANCA associated vasculitis
necrotising polyangiitis that affects capillaries, venules and arterioles
symptoms of ANCA vasculiitis
- fever
- migratory arthralgia
- weight loss
- anorexia
- malaise
diagnosis of ANCA vasculiitis
- urinalysis - active urine, blood and protein
- bloods - raised inflammatory markers, AKI, anaemia
- immunology - ANCA, anti-MPO, anti-PR3
- renal biopsy - crescentic GN, pauci-immune
what are the three types of ANCA vasculiitis
- granulomatosis with polyangiitis (Wegener’s granulomatosis)
- Microscopic polyangiitis (MPA)
- eosinophilic granulomatosis with polyangiitis (churg-strauss)
what is GPA
- predominantly anti-PR3 antibodies
- necrotising granulomatous inflammation
- lung involvement (pulmonary/renal syndrome)
what is microscopic polyangitis
- anti-MPO antibodies
- small cell vasculitis with no granulomas
- systemic features/renal/lung/skin/GI/nerves
