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Yr 3 Infection > vasculitides > Flashcards

vasculitides Flashcards

1
Q

definition of vasculitides

A

vasculitis is the inflammation adn necrosis of blood vessels

primary vasculitides are classified according to the main vessel size effected

large = GCA, Takayasu’s aortitis

medium = polyarteritis nodosa, Kawasaki’s disease

small = Churg-Strauss syndrome (CSS), microscopic polyangitis (MP), Henoch-Schonlein purpura (HSP), Wegener’s granulomatosis (WG), mixed essential cryoglobulinaemia (MEC), relapsing polychondritis (RP)

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2
Q

what are secondary vasculitides

A

vasculitis occuring secondary to infections, abscesses, malignancies adn connective tissue disease (RA, SLE)

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3
Q

aetiology of vasculitides

A

unknown

thought to be autoimmune

immune complex deposition in vessel walls triggers classical complement activation and inflammation

small vessel are ANCA associated vasculitis - ANCA activate the neutophils = local inflammation and vasculitis

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4
Q

pathology of vasculitidesn

A

acute and chronic inflam cells in vessel wall

some subtypes (GCA, WG, CS and PAN) have evidence of granulomatous inflammation

PAN affect medium sized arteries

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5
Q

RF for vasculitides

A

PAN associated with Hep B

MEC with Hep C

MP associated with presence of pANCA

WG is associated with c-ANCA

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6
Q

epidemiology of vasculitides

A

annual incidence of small vessel vasculitis is 1 in 10000

TA is more common in Japanese young females

PAN can affect any age - female more

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7
Q

sx and signs of all vasculitides

A

large vessel have classical patterns from the vessels affected

medium and small - characterised by multiorgan involvement with less specific clinical features

general - fever, night sweats, malaise, weight loss, myalgia

skin - rash (vasculitic, purpuric, maculopapular, livedo reticularis) nailbed infarcts, digital gangrene

joint - arthralgia, arthritis

GI - abdo pain, haemorrhage from mucosal ulceration, diarrhoea, perforation (infarcted viscus), malabsorption (chronic ischemia)

kidney - glomerulonephritis, renal failurel HTN, haematuria, proteinuria, casts, renal failure (renal cortical infarcts)

lung - dyspnoea, cough, chest pain, haemoptysis, lung haemorrhage

CVS - pericarditis, coronary arteritis -> MI, myocarditis, HF, arrhythmias, angina,

CNS - mononeuritis mulitiplex, infarctions, meningeal involvement

eyes - retineal haemorrhage, cotton wool spots, episcleritis, scleritis, vision loss

ENT - epistaxis, nasal crusting, stridor, deafness

neuro - stroke, fits, choream psychosis, confusion, impaired cognition, altered mood, arteritis of the vasa nervorum (arterial supply to the peripheral nerves) may cause mononeuritis multiplex or a sensorimotor polyneuropathy

GU - orchitis-testicular pain or tenderness

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8
Q
A

livedo reticularis

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9
Q

sx of Takayasu’s aortitis

A

constitutional upset

head or neck pain

tenderness over affected arteries (aorta and the major branches)

dizzyness

fainting

low peripheral pulses

hypertension

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10
Q

sx and signs of polyarteritis nodosa

A

microaneurysms

thrombosis

infarctions eg causing GI perf

hypertension

testicular pain

skin - rash, punched out ulcers, nodules

renal is main cause of death - renal artery narrowing, glomerular ischemia, insufficiency

cardiac, GI, GU and neuro involvement

mononeuritis multiplex

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11
Q

sx and signs of Kawasaki disease

A

age <5yr

fever >5 days

fissured lips

red swollen palms and soles followed by desquamation

skin rash

inflammed oral cavity

conjunctival congestion

lymphadenopathy

coronary artery aneurysm

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12
Q

sx and signs of churg-strauss syndrome

A

asthma

eosinophilia

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13
Q

signs and sx of henoch-schonlein purpura

A
  • triggered by URTI - affects children>adults*
  • IgA immune complexes deposit in the small vessels*

purpura of leg and buttocks

arthritis

gut symptoms - abdo pain

glomerulonephritis with IgA deposition

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14
Q

signs and sx of microscopic polyangitis

A

non-specific with multiple organs affected

glomerulonephritis with no glomerular Ig deposits

pulmonary haemorrhage in up to 30%

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15
Q

signs and sx of wegener’s granulomatosis/granulomatosis with polyangitis

A

granulomatous vasculitis of upper and lower resp tract

nasal discharge

ulceration and deformity, saddle nose

heamoptysis

sinusitis

corneal thinning

glomerulonephritis

nodules and pulmonary haemorrhage

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16
Q

signs and sx of relapsing polychondritis

A

affecting cartilage (eg ear pinna, nose, larynx) causing swelling, hoarse voice, tenderness, cartilage destruction and deformity eg saddle nose

17
Q

signs and sx of mixed essential cryoglobinaemia

A

arthritis

splenomegaly

skin vasculitis

renal disease

cryoglobins (IgG and IgM mix)

18
Q

Ix for vasculitides

A
  • blood
    • FBC - normocytic anaemia, high platelets and neutrophils
    • high ESR/CRP
    • creatinine - high if renal failure
  • autoAb
  • urine
    • haematuria
    • proteinuria
    • red cell casts
  • CXR
    • diffuse, nodular or flitting shadows
    • atelectasia
  • biopsy
    • renal
    • lung (transbronchial)
    • temporal artery in GCA
  • angiography
    • to identify aneurysms in PAN
19
Q

auto-antibodies looked for in vasculitides

A

c-ANCA: anti-proteinase 3 associated with WG

p-ANCA: anti-myeloperoxidase seen in MP and CSS, but also in IBD, primary biliary cirrhosis and chronic active hepatitis

ANA, anti-dsDNA may suggest SLE

Rheumatoid factor - +ve in 50%

cryoglobulins (immunoglobulins and complement components that precipitate at temp <37 degrees)

20
Q

definition of polyarteritis nodosa

A

necrotising vasculitis

cayses aneurysms and thrombosis in medium sized arteries

leading to infarction in affected organs = systemic sx

demalre more

associated with Hep B

rare in UK

21
Q

Ix for PAN

A

high WCC

mild eosinophilia

anaemia

high ESR and CRP

ANCA -ve

renal or mesentaric angiography or renal biopsy can be diagnostic

22
Q

define microscopic polyangitis

A

necrotising vasculitis affecting small and medium vessels

23
Q

Ix for microscopic polyangitis

A

ypANCA (MPO) +ve

24
Q

where are the vessels that PAN effects

A

GIT

coronary

renal

skin - palpable nodules

nerves - mononeuritis neuroplex

25
Q

what has an association with PAN

A

hep B

26
Q

pathophysiology of immune complex vasculitis

A

immune complex formation

complement activation

infiltration of macrophages and neutrophils

cytokines and chemokines expression

granule release from neutrophils

increased vascular permeability

inflammation and damage to vessels

Yr 3 Infection (25 decks)

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