vasculitides Flashcards
definition of vasculitides
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)
what are secondary vasculitides
vasculitis occuring secondary to infections, abscesses, malignancies adn connective tissue disease (RA, SLE)
aetiology of vasculitides
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
pathology of vasculitidesn
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
RF for vasculitides
PAN associated with Hep B
MEC with Hep C
MP associated with presence of pANCA
WG is associated with c-ANCA
epidemiology of vasculitides
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
sx and signs of all vasculitides
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
livedo reticularis
sx of Takayasu’s aortitis
constitutional upset
head or neck pain
tenderness over affected arteries (aorta and the major branches)
dizzyness
fainting
low peripheral pulses
hypertension
sx and signs of polyarteritis nodosa
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
sx and signs of Kawasaki disease
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
sx and signs of churg-strauss syndrome
asthma
eosinophilia
signs and sx of henoch-schonlein purpura
- 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
signs and sx of microscopic polyangitis
non-specific with multiple organs affected
glomerulonephritis with no glomerular Ig deposits
pulmonary haemorrhage in up to 30%
signs and sx of wegener’s granulomatosis/granulomatosis with polyangitis
granulomatous vasculitis of upper and lower resp tract
nasal discharge
ulceration and deformity, saddle nose
heamoptysis
sinusitis
corneal thinning
glomerulonephritis
nodules and pulmonary haemorrhage
signs and sx of relapsing polychondritis
affecting cartilage (eg ear pinna, nose, larynx) causing swelling, hoarse voice, tenderness, cartilage destruction and deformity eg saddle nose
signs and sx of mixed essential cryoglobinaemia
arthritis
splenomegaly
skin vasculitis
renal disease
cryoglobins (IgG and IgM mix)
Ix for vasculitides
- 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
auto-antibodies looked for in vasculitides
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)
definition of polyarteritis nodosa
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
Ix for PAN
high WCC
mild eosinophilia
anaemia
high ESR and CRP
ANCA -ve
renal or mesentaric angiography or renal biopsy can be diagnostic
define microscopic polyangitis
necrotising vasculitis affecting small and medium vessels
Ix for microscopic polyangitis
ypANCA (MPO) +ve
where are the vessels that PAN effects
GIT
coronary
renal
skin - palpable nodules
nerves - mononeuritis neuroplex
what has an association with PAN
hep B
pathophysiology of immune complex vasculitis
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
