Kidney in Systemic Disease Flashcards
What is dysproteinaemia
Abnormal proteins
Dysregulation leads to overproduction
usually from the B cell line
What is myeloma
Cancer of the plasma cells
The abnormal cells accumulate in the marrow and impair production of normal blood cells
They also overproduce abnormal antibodies - these block the kidneys
How does myeloma present
Bone Pain - particularly back Renal failure Weakness Fatigue Weight Loss Recurrent infections Anaemia Hypercalcemia Lytic bone lesions
How does myeloma affect the kidneys
Deposits of protein in the glomerulus
Can get light chain cast nephropathy - lumps of protein visible in tubules
Which tests should you do if myeloma is suspected
Serum protein electrophoresis Serum free light chains Urine - BJ proteins Bone marrow biopsy Skeletal survey Renal biopsy
How do you treat myeloma
Stop nephrotoxics Manage hypercalcaemia (saline +/- bisphosphonates) Chemo Stem cell transplant is curative May use plasma exchange or dialysis
What is amyloidosis
Deposition of extracellular amyloid in tissue/organs
Due to abnormal folding of proteins which makes them insoluble and they aggregate
What are the 4 most common types of amyloidosis
Primary/light chain (AL)
Secondary/inflammatory (AA)
Dialysis
Hereditary and old age
Describe AL (primary light chain) amyloidosis
Caused by overproduction of light chains – small bits of antibody
Get into blood and form deposits
Describe AA (secondary) amyloidosis
Associated with systemic inflammation
Production of acute phase protein - SAA
Seen in chronic inflammatory conditions - RA, IBD
Where does AL (primary) amyloidosis affect
Heart Bowel Skin Nerves Kidneys
Where does AA (secondary) amyloidosis affect
Liver
Spleen
Kidneys
Adrenals
How does amyloidosis present
depends on the organs involved Get proteinuria and impaired renal function Cardiomyopathy Neuropathy Hepato/splenomegaly Malabsorption
How do you diagnose amyloidosis
Urinalysis and urine PCR
Bloods - renal function, inflammatory markers, electrophoresis
Renal biopsy - need a tissue diagnosis to confirm
SAP scan
How do you manage AA amyloidosis
treat the underlying inflammatory condition
refer to national centre at UCL
How do you treat AL amyloidosis
Immunosuppression
Steroids, chemotherapy, stem cell transplant
Refer to national centre at UCL
How do you stain a renal biopsy for amyloidosis
Use Congo red stain
Will give apple green bifringence
How does small vessel vasculitis present
Fever, migratory arthralgia, weight loss, anorexia and malaise
How do you diagnose ANCA vasculitis
Urinalysis - blood and protein
Antibody tests - ANCA etc
Bloods - inflam markers, AKI etc
How do you manage vasculitis
Immunosuppression:
Steroids
Cyclophosphamide or rituximab
Plasma exchange
Supportive - dialysis etc
Describe GPA
Predominantly anti-PR3
Necrotising granulomatous inflammation
Lung involvement as well
as kidney
Describe microscopic polyangiitis
Anti-MPO
Small vessels with no granulomas
Systemic symptoms - renal, lung, skin, GI etc
Describe eosinophilic GPA
Associated with asthma and eosinophilia
2/3 have skin involvement
How does lupus nephritis present
Occurs in 50% of lupus patients
Proteinuria
Many different classifications
How do you differentiate between the classes of lupus nephritis
Renal biopsy is the only way
