Kidneys in Systemic Disease Flashcards
what is dysproteinaemia
overproduction of immunoglobulin by clonal expansion of cells from B cell lineage
what is the main role of plasma cells
to make antibodies
what is myeloma
cancer of the plasma cells
causes excess production of immunoglobulins
what happens in myeloma
- collecting of abnormal plasma cells accumulate in the bone marrow
- impairment of production of normal blood cells
- monoclonal production of a paraprotein
- can cause renal dysfunction
what are the symptoms of myeloma
bone pain weakness fatigue weight loss recurrent infections
classic presentation= back pain and renal failure (AKI)
what are the signs of myeloma
anaemia
hypercalcaemia
renal failure
lytic bone lesions
what are the renal manifestations of myeloma
20-40% present with renal impairment
glomerular: AL amyloidosis, monoclonal immunoglobulin deposition (light/heavy chains)
tubular: light chain cast nephropathy
dehydration. hypercalcaemia
what is myeloma kidney
cast nephropathy (free light chains form waxy casts within the tubule lumen)
how do you diagnose myeloma
have high index of suspicion bloods: -serum protein electrophoresis (measures globulins) -serum free light chains urine: -bence jones protein
bone marrow biopsy
skeletal survey
renal biopsy (rarely done)
what is the management for myeloma
stop nephrotoxics and manage hypercalcaemia (saline +/- bisphosphonates)
CHEMOTHERAPY (majority of patients get this)
stem cell transplant
plasma exchange (to remove light chains)
supportive= dialysis
what is amyloidosis
deposition of extracellular amyloid (insoluble protein fibrils) in tissues and organs
why are amyloid proteins deposited
because the are abnormally folded which causes them to aggregate and become insoluble
(normal degradation pathways break down)
what are the 4 most common types of amyloidosis
primary/ light chain (AL)
secondary/ systemic/ inflammatory (AA)
dialysis (Abeta2M)
hereditary and old age (ATTR)
what is AL amyloidosis
unknown causes causes production of abnormal immunoglobulin light chains from plasma cells
(light chains enter the blood stream and cause amyloid deposits)
what is the usual presentation of AL amyloidosis
commonly affects heart, bowel, skin, nerves and kidneys
age at diagnosis 55-60 life expectancy (untreated)= 6 months- 4 years
what is AA amyloidosis
associated with systemic inflammation
production of acute phase protein - serum amyloid A protein (SAA)
what is the presentation of AA amyloidosis
develops in people with chronic inflammatory conditions/ chronic infections
- RA, IBD, psoriasis
- TB, osteomyelitis, bronchiectasis
commonly affects liver, spleen, kidneys and adrenals
what are the possible presentations of amyloid
depends on the tissues affected:
- renal= nephrotic range proteinuria +/- impaired renal function
- cardiac= cardiomyopathy
- nerves= peripheral/ autonomic neuropathy
- hepatomegaly/ splenomegaly
- GI= malabsorption
what investigations for amyloidosis
urinalysis + uPCR
bloods- renal function, markers of inflammation, protein electrophoresis, SFLC (serum free light chains)
renal biopsy (congo red staining- apple green under polarised light)
can do abdo fat/ rectal biopsy
SAP scan shows extent of the disease
what do you need to diagnose amyloidosis
tissue biopsy (congo red stain- apple green bifringence)
what is the management for amyloidosis
not curative
-reduce further deposition and preserve organ function
AA= treat underlying condition
AL= immunosuppression (steroids, chem, stem cell transplant)
what is vasculitis
inflammation of the blood vessels
what are the ANCA associated vasculitis
MPA
GPA
eGPA
drug induced
what is small vessel ANCA associated vasculitis
necrotising polyangitis that affects capillaries, venules and arterioles
what are the systemic symptoms of small vessel ANCA associated vasculitis
fever, migratory arthralgia, weight loss, anorexia, malaise
what age group is small vessel ANCA vasculitis most common
5th, 6th and 7th decade
how do you diagnose ANCA vasculitis
high index of suspicion
urinalysis- blood ++ protein ++
raised inflammatory markers, AKI, anaemia
ANCA
eGPA + MPA = anti MPO + pANCA
GPA= cANCA + PR3
renal biopsy
what is GPA
necrotising granulomatous inflammation
-anti- PR3 antibodies
where does GPA affect
lung (pulmonary/ renal syndrome), nose, nasal symptoms, epistaxis, saddle nose
what is MPO
small vessel ANCA vasculitis with no granulomas
- anti MPO antibodies
- systemic features: renal, lung, skin, GI, nerves
what is eGPA
associates with late onset asthma and eosinophilia
-2/3rds have skin involvement
what is the management for vascultitis
immunosuppression
- steroids
- cyclophosphamide/ rituximab
plasma exchange
supportive- dialysis, ventilation
what are 2 features of the malar rash in SLE
butterfly shaped
photosensitive
what is SLE
chronic autoimmune inflammatory disease (unknown origin)
affects skin, joints, kidneys, lungs, nervous system, serous membranes
who gets SLE
women (10:1) in theirs 20-30s
african americans and hispanics better risk
how do you diagnose SLE
high index of suspicion in young lady with non specific symptoms
bloods:
-raised inflammatory markers
-immunology- ANA +ve, anti-dsDNA
complete (low)
urinalysis
what can be used to measure disease activity in SLE
anti-dsDNA and complement (low when disease high)
what is lupus nephritis
renal involvement in SLE
most frequently observed abnormality is proteinuria
what are the types of lupus nephritis
classified on how advanced class I minimal mesgangial class VI advanced sclerosing determines management
what is the prognosis of SLE
varied clinical course
relasping/ remitting
poor prognostic factors for survival
- renal disease
- male sex
- young/ old at presentation
- deprivation
- antiphospholipid syndrome
- high disease activity
what are the systemic symptoms of SLE
fever weight loss arthralgia rash proteinuria pleuritis
what is the general management for SLE
immunosuppression
what is required in suspected lupus nephritis
renal biopsy
