R: Vasculitis Flashcards
what is vasculitis
umbrella term for inflammation of vessel walls
what are the 2 features used to classify vasculitides?
size of blood vessels & presence of anti-neutrophil cytoplasmic antibodies (ANCA) in blood
what do we mean by large vessel?
aorta & its tributaries
pathophysiology of vasculitides?
dendritic cells activated releasing inflammatory cytokines > t-cells activated promoting granuloma formation & macrophage activation > mediators released causing vascular inflammation, endothelial damage etc
Polymyalgia reumatica and giant cell arteritis are a…
large vessel vasculitis
what are Polymyalgia reumatica and giant cell arteritis
granulomatous infiltrations of walls of large vessels
- GCA: arteritis of large cerebral arteries
s/s of Polymyalgia reumatica and giant cell arteritis
PMR: non-specific: fever, malaise, fatigue, hip/bum/thigh pain and stiffness that is worse in morning
GCA: headaches, tenderness of scalp, jaw claudication, visual loss, tenderness of occipital arteries
ix for Polymyalgia reumatica and giant cell arteritis
bloods: inflammatory markers
LFTs: ALP & gamma GT raised
temporal artery biopsy
MR Angio *may show thickened vessel walls)
mx of Polymyalgia reumatica and giant cell arteritis
low dose long term steroids + steroid sparing agents (MTX)
what is another large vessel vasculitis more common in females and asian population?
Takayasu’s Arteritis
what is a medium vessel vasculitides?
polyarteritis nodosa/ Kawasaki’s disease
epi for polyarteritis nodosa/ Kawasaki’s disease
PN: middle aged men
Kawasaki’s: children <5
aetiology of polyarteritis nodosa/ Kawasaki’s disease
PN: hepB?
Kawasaki’s: infective cause
pathophysiology of polyarteritis nodosa/ Kawasaki’s disease
fibrinoid necrosis of vessel walls with micro aneurysm formation, thrombosis, infarction
s/s of polyarteritis nodosa/ Kawasaki’s disease
PN: fever/wt loss
- neuro: mononeuritis multiplex
- renal: haematuria/ proteinuria
cardiac: coronary arteritis
kawasakis: fever >5days, conjunctival congestion 2-4days after onset, dry/red oral cavity, cervical LN
ix of polyarteritis nodosa/ Kawasaki’s disease
PN: bloods, biopsy, angio, screen fro hepB, urinalysis
K: fever + 4/5 of other s/s + inc CRP
mx for polyarteritis nodosa/ Kawasaki’s disease
PN: corticosteroids + immunosuppression
K: immunoglobulin & aspirin
how are small vessel vasculitides divided?
ANCA +Ve & ANCA -ve
What are the anca +ve small vessel vasculitides?
granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis
T/F: Wegner’s is another name for EGPA?
F: Wegner’s is another name for GPA
epi for GPA & EGPA?
GPA: late adulthood
EGPA: early adulthood
s/s for GPA & EGPA?
GPA: fever, arthralgia
- ENT*: bloody nasal discharge, nasal discharge, otitis media
- renal: necrotising glomerulonephritis
- resp: cavities on x-ray, cough, pleuritic pain
- cutaneous: non-blanching purpura rash
neuro: CN palsies, mononeuritis multiplex
EGPA: triad of rhinitis, late onset asthma that is difficult to control, raised peripheral blood eosinophilia
ix for GPA/EGPA?
GPA: biopsy*, bloods, urinalysis
- serology: c-ANCA +ve, inc PR3 antibodies
EGPA: biopsy, x-ray for pulmonary infiltrates
- serology: p-ANCA +ve
mx for GPA/EGPA?
GPA: glucocorticoids & DMARDs
RGPA: corticosteroids + DMARDs
- DMARDs= stronger given for more severe disease e.g. localised= MTX, generalised= rituximab
golemurlonephritis is a common complication of another medium vessel vasculitis, what is this?
microscopic polyangiitis
what are the 2 ANCA -ve small vessel vasculitides?
Henoch-Schonlein Purpura and Goodpasture’s syndrome
what is HSP?
acute IgA mediated vasculitis involving GI, kidneys, joints and skin
epi for HSP?
2-11 yo
aetiology of HSP?
often follows an URTI, pharyngeal or GI infection
what is the causative organism for HSP?
group A streptococci
s/s of HSP?
purpuric skin rash on buttocks/ lower limbs, abdominal colic & bloody diarrhoea/ vomiting, glomerulonephritis
mx for HSP?
self-liming (8weeks)
what is the triad for Goodpasture’s syndrome?
pulmonary haemorrhage, glomerulonephritis, antibody presence to basement membrane of kidneys & lungs
mx for GPS?
plasmapheresis and immunosuppression
what is Behcet’s vasculitis?
chronic AI vasculitis which characteristically presents with recurrent oral and genital ulcers
T/F: Behcet’s is a treatable condition
T but it is a relapsing-remitting condition
which gene may be implicated and is a good indicator for severity in Behcet’s?
HLA B51
s/s of Behcet’s affects the whole body- from lungs (pulmonary artery aneurysm) to CNS (memory impairment), but what is the classical triad?
oral ulcers, genital ulcers, uveitis
what is the Ix for Behcet’s?
pathergy test- wheal >55mm formation with subcutaneous abrasion
mx for Behcet’s?
combo of steroids & colchicine
