The kidney in systemic disease 2 Flashcards
What is multiple myeloma ?
- It is a cancer of plasma cells (a type of ABC responsible for producing antibodies)
- A collection of abnormal plasma cells accumulate in the bone marrow, where they interfere with production of normal blood cells
- Most cases also feature the production of a paraprotein (an abnormal antibody which can cause kidney problems)
What are the signs and symptoms of multiple myeloma ?
- Bone pain
- Weakness/fatigue
- Weight loss
- Anaemia
- Hypercalcaemia
- Renal failure
- Amyloidosis
- Recurrent infections
What are some of the investigations used to detect multiple myeloma ?
- Serum/urine electrophoresis - this shows paraprotein spike and light chain urinary excretion
- Bence Jones protein in urine
What are the renal manifestations of myeloma ?
- AKI secondary to hypercalcaemia
- Amyloidosis
- Cast nephropathy
What is the treatment of multiple myeloma with ARF?
- Stop nephrotoxins (e.g. NSAID’s & diuretics)
- Treat hypercalcaemia - give IV NaCl to increase volume and IV pamidronate if required
- Chemo to reduce tumour load
- Dialysis to support AKI and CKD
What type of vasculitis mainly affect the kidneys?
ANCA associated small vessel vasculitis
What are the 3 ANCA vasculitis which can affect the kidneys ?
- GPA (wegners)
- MPA
- Churg-strauss (EGPA)
What are the general presenting features of vasculitis ?
- Fever & weight loss
- Raised non-blanching rash
- Arthralgia/arthritis
- GN
- Lung opacities on X-ray
Define what GPA (wegners) is
- It is a granulomatous inflammation involving the resp tract
- GN is commonly seen
What are the common features associated with GPA (wegners)?
ENT symptoms:
- Nose bleeds
- Deafness
- Recurrent sinusitis & nasal crusting
- May be collapse of the nose (saddle shaped nose)
Resp symptoms:
- Haemoptysis & cavitating lesions seen
What antibodies is GPA (wegners) associated with ?
cANCA & PR-3
What is churg strauss (EGPA)?
- It is a granulomatous inflammation of vessels involving the resp tract
- Characterised by asthma, rhinitis & eosinophilia
- Infrequently causes GN
What is MPA ?
- It is a necrotising vasculitis with few or no immune deposits (no granulomatous inflammation)
- GN occurs in up to 90% of patients
What antibody are EGPA (churg strauss) and MPA associated with ?
MPO-ANCA
What investigations are done to diagnose vasculitis which affect the kidneys ?
- Bloods - show raised ESR,PV & CRP, ANCA present
- CXR - for lung opacities
- Biopsy affected area e.g. skin or kidney
What is the management of these vasculitis ?
IV steroids and cyclophosphamide
What is amyloidosis ?
- Generic name for a family of diseases
- Characterised by deposition in extracellular spaces of a proteinaceous material
What is the classic histological appearance of amyloidosis ?
Positive Congo red staining showing apple-green birifringence under polarised light
What is SLE ?
It is a chronic autoimmune disease affecting the skin, joints, kidneys, lungs and nervous system
Who is most commonly affected by SLE ?
Women in there 20s and 30s
What are the antibodies specific to SLE ?
Anti-ds antibody and Anti-sm
What is the characteristic rash seen in SLE ?
Malar rash
What renal problem can SLE cause
GN
What is the treatment of lupus nephritis ?
Induction:
- non immunosuppressive - ACEi/ARB for BP
- Immunosuppressive - high dose steroids + cyclophosphamide + azathioprine
Maintenance:
- Steroids + azathioprine
