Glomerulonephritis and PCKD Flashcards
what is interstitial nephritis
inflammation of space between cells and tubules = interstitium
what is glomerularsclerosis
pathological process of scarring of tissue in glomerulus, caused by GN or obstructive uropathy
what is IgA nephropathy
bergers disease = most common cause of glomerulonephritis
- peak age: 20
IgA deposits in mesangium
overalps with HSP
how does IgA nephropathy present and how is it managed?
classically: macroscopic haematuria in young people following an UPPER RESP TRACT INFECTION
manage with monitoring 6-12 months, ACE-I if hypertensive
what is nephrotic syndrome?
when basement membrane in glomerulus becomes highly permeable to protein so leaks into urine
damaged podocytes, so loss of albumin (main protein that keeps blood in blood vessels)
low albumin = increased lipids by liver
loss of anti-thrombin 3= hypercoaguable
what is the classic triad of nephrotic syndrome?
low serum albumin
high urine protein +3 on dipstick
oedema
= also may have hypercholesterolaemia
what causes nephrotic syndrome?
minimal change disease in children
in adults: focal segmental glomerularsclerosis (presents in young adults, due to idiopathic reasons, HIV, renal path, heroin etc)
secondary causes: HSP, diabetes, infection
how do you manage nephrotic syndrome?
high dose steroids for 4 weeks low salt diet diuretics fluid restriction: 1-1.5L albumin infusions antibiotic prophylaxis anti-coag?
what is nephritic syndrome?
inflammation of kidney = group of symtpoms
antibody meets antigen to form immune complex
this complex will lodge itself in capillary and cause an IR against capillary and antigens
WCC are recruited (hence on FBC, high WCC)
Areas become inflamed, and breakdown
Allows RBC and WBC to pour through the opening
• Hence protein gets through as well
what are the features of nephritic syndrome?
peripheral oedema
proteinuria
serum albumin <25
oliguria
high BP
granular casts
what would you see on a urine dip for nephritic?
proteins, bloods, WC
what are the causes of nephritic syndrome?
minimal change disease
focal segmental
primary: IgA, post-strep GN, GPS
main difference between nephritic and nephrotic?
only protein in urine = nephrotic
protein, sediments and extra cells = nephritic
what is glomerulonephritis, and what are some examples?
conditions that cause inflammation of or around glomerulus and nephron
o Minimal change disease o Focal segmental glomerulosclerosis o Membranous glomerulonephritis o IgA nephropathy (AKA mesangioproliferative glomerulonephritis or Berger’s disease) o Post streptococcal glomerulonephritis (AKA diffuse proliferative glomerulonephritis) o Mesangiocapillary glomerulonephritis o Rapidly progressive glomerulonephritis o Goodpasture Syndrome
what is the pathophysiology of GN
immunoglobulin and complement activation within glomeruli
antigens may be trapped and deposited
what is membranous GN
most common type
- ages 20 and 60
IgG deposits in basement membrane
idiopathic
secondary to malignancy, NSAIDs, rheumatic disorders
management: ACE-I, immunosuppression if svere (ccs and cyclophosphamide)
what is the prognosis of membranous GN
o 1/3 recover spontaneously, 1/3 remain proteinuric, 1/3 develop ESRF
what is membranoproliferative GN
= same as membranous but deposits also in mesangium
what is post-strep GN?
patients <30 years presents as: - 1-3 weeks after a strep infection -develops as acute nephritic syndrome - malaise, haematuria, proteinuria, hypertension, oliguria
caused by: immune complex depositition in glomeurli
what causes post-strep GN and what are the investigations?
immune complex deposition in glomeruli
- low C3 on bloods, renal biopsy, starry sky appearance
what is GPS?
small cell vasculitis
- anti-GBM antibodies attack glomerulus and pulmonary basement membrane
= GN and pulmonary haemorrhage
- more common in men, age 20 and 60
what are the tests and management for GPS
linear IgG deposits and CO transfer factor is raised
management: plasma exchange, steroids, cyclophosphamide
what differential could you give for GPS
wegeners (ANCA vasculitis may present with wheeze and sinus)
what is rapidly progressive GN
very acute illness w sick patients
secondary to GPS often
nephritic syndrome features
treated with BP control and steroids
what is vasculitis
inflammation of the blood vessles, diff sizes
what type of vasculitis affects small vessels?
Henoch=schonlein purpura
Eosinophilic granulomaosis with polyangiitis (churg-strauss syndrome)
Microscopic polyangiitis = main feature is renal failure
Granulomatosis with polyangiitis (wegener’s granulomatosis)
what type of vasculitis affects medium sized vessels?
Polyarteritis nodosa
Churg-strauss syndrome
Kawasaki disease
what type of vasculitis affects large vessels?
GCA and takayasu’s arteritis
what is the main complication of GCA?
blindness
what are some generic presentations of vasculitis?
purpura, joint and muscle pain peripheral neuropathy renal impairment anterior uveitis and scleritis GI disturbance and bleeding hypertension
what are the systemic manifestations of vasculitis
fever, fatigue, WL, anaemia
how do you test for vasculitis?
CRP and ESR raised
ANCA blood test
what is HSP?
IgA vasculitis = affects children
presents as rash on legs and bum
inflammation bc of IgA deposits on BV of affected organs
often triggered by URTI
most common in children under age of 10
rash due to leaking from small BV
what are the features of HSP and how do you treat it?
purpura, joint pain, abdo pain, renal involvement
= analgesia and hydration
explain the ANCA system
Antineutrophil cytoplasmic antibodies (ANCA) are a group of autoantibodies produced when a person’s immune system mistakenly targets and attacks its own neutrophil proteins. Two of the most commonly targeted proteins are myeloperoxidase (MPO) and proteinase 3 (PR3)
what are p-anca and c-anca?
p-ANCA: also called anti-MPO antibodies –> microscopic polyangiitis and churg-strauss syndrome
typical pattern of peri-nuclear staining, specific for myeloperoxidase enzyme
c-ANCA also called anti-PR3 antibodies –>
wegener’s granulomatosis
diffuse cytoplasmic staining
both enzymes are stored in neutrophil granules
what is churg strauss syndrome?
small and medium vasculitis
lung and skin problems
presents with severe asthma in late teens
eleveated eosinophils on FBC
what is granulomatosis with polyangitis?
wegeners
CLASSIC SIGN: saddle shaped nose
small vessel
affects resp and kidneys (epistaxis, crusty secretions), cough, wheeze, haemoptysis with consolidation on CXR
leads to rapidly progressing GN
what is the pathogenesis of ANCA vasculitis
expression of auto-antigeon on surface due to cytokines
binding = activation of neutrophils
cytotoxic injury to vessel wall
apoptosis and necrosis of neutrophils and endothelial cells
how do you diagnose ANCA vasculitis?
history and examination, ANCA blood test, kidney biopsies, CXR, CO transfer factor differentiates between pulm oedema and presence of blood in alveoli
how do you manage ANC vasculitis?
Induction of remission: cyclophosphamide and steroids
Maintenance of remission: azathioprine & CCS
what is polyarteritis nodosa?
Medium vessel
Most associated with hep B = clear cause or hep C and HIV
Affects skin, GI tract, kidneys and heart
Renal impairment, strokes and myocardial infarction
Usually ANCA negative
how does PAN present and how is it treated?
Presentation: GI bleed, bowel infarction, HTN, renal artery aneurysms
Livedo reticularis = mottled, purplish, lace like rash Diagnosis: angiogram
Treatment: if hep B neg, give CCS and cyclophosphamide
If hep b positive: antiviral and CCS
what is kawasaki disease?
Medium vessel vasculitis
Common in oriental world
• Young children <5 years
Clinical features:
-Persistent high fever >5 days
-Erythematous and desquamation of palms and soles
-Strawberry tongue (red tongue with prominent papillae)
how do you treat kawasaki disease and what is the key complication?
Treatment: symptomatic but IV immunoglobulins are used for severe form
Key complication = coronary artery aneurysm
what is takayasu’s arteritis?
large vessel
aorta, pulmonary arteries
can swell and form aneurysms or can become narrow and blocked
= AKA pulseless disease
how does takayasus present and how is it treated?
fever, malaise, muscle aches, claudication or syncope
CT, MRI angio or doppler of US carotids
treatment: steroids
what is polycystic kidney disease
genetic condition where kidneys develop multiple fluid filled cysts
two types are autosomal dominant and autosomal recessive
how does ADPKD present?
= chromosome 16 or 4
- extra renal: cerebral aneurysms, cysts on liver, spleen, pancreas etc
- issues with cardiac valve and colonic diverticula
what is the USS diagnostic criteria for ADPKD?
Two cysts, unilateral or bilateral, if aged <30 years
2 cycts in BOTH kidneys if 30-59
4 cysts in both kidneys if aged >60 years
- Also, use CT scans to diagnose
what are complications of ADPKD?
Chronic loin pain Hypertension Cardiovascular disease Gross haematuria if cyst ruptures Renal stones (common in patients with PKD) ESRD at age 50
what is ARPKD?
gene on chromosome 6
rarer and more severe
presents in childhood/infancy
= presents in pregnancy with oligohydraminos as fetus doesn’t produce enough urine
how else does ARPKD present?
- may require dialysis in first few years of life
dysmorphia = ears are low set, flat nasal bridge
ESRD before adulthood
what does a renal biopsy show for ARPKD?
multiple cylindrical lesions at RA to cortical surface
how do you manage polycystic kidney disease?
tolvaptan = vasopressin receptor antagonist
slows the development of cysts and progression of RF if CKD stage 2 or 3
- nephrectomy if poor prognosis otherwise
supportive: anti-hypertensivem analgesia, AB, drainage of INFECTED cysts
what is von hippel lindau disease?
autosomal dominant condiiton on chromosome 3 (encodes TSG protein)
presents in 20s or 30s with malignancies in retina, CNS, kidneys
renal manifestations: cortical renal cysts, renal cell carcinoma
treatable by surgical intervention
screening in genotype confirmed patients is annual USS and CT every 3 years (for fam members CT too)
what are simple cysts
non genetic cause of cystic disease
more common with age
Unilocular and located in the cortex
Kidneys do not get enlarged by these cysts
Over the age of 30
Unlikely that on its own this will cause haematuria or pain
what is acquired renal cystic disease?
Patients who have had a long duration of dialysis can develop acquired renal cysts
Tend to have shrunken kidneys and a negative family history of cystic diseases
