MSK- vasculitis Flashcards
two major categories of large vessel vasculitis
temporal (giant cell) arteritis and Takayasu arteritis (TA)
Takayasu arteritis aetiology
<40 years
females
Asian
CF Takayasu arteritis
systemic- weight loss, night sweats, fever, malaise
claudication in upper and lower limbs
CF Takaysu arteritis- if left untreated
vascular stenosis and aneurysms- carotid bruit, reduced pulses, bp differences extremities
giant cell arteritis age range
> 50, most commonly late 60s
giant cell arteritis associated with
polymyalgia rheumatica
GCA typical presentation
new-onset headache in patients 50 years of age or older with an elevated erythrocyte sedimentation rate (ESR), CRP or plasma viscosity
GCA symptoms
unilateral acute temporal headache with focal tenderness on direct palpation
jaw claudication on chewing firm foods or speaking
visual disturbances
visual loss
constitutional manifestations
GCA signs
Tender enlarged non-pulsatile temporal arteries
first line investigation in GCA
temporal artery USS
gold standard investigation in GCA
temporal artery biopsy
management of large vessel vasculitis
Start at prednisolone 40-60mg daily
TA specific management
more long term, use steroid sparing agents if needed e.g. leflunamide, methotraxate
GCA specific management
gradual reduction in steroid dose over 18 months to 2 years
GCA treatment with visual loss
IV methylprednisolone 500mg
GCA treatment for bone protection
bisphophonate
vitamin d
calcium
polyarteritis nodosa most commonly affects which sex and age group
men
aged 40-60
polyarteritis nodosa associated with
hepatitis b
skin symptoms of polyarteritis nodosa
livedo reticularis, nodules, ulcers, palpable purpura
polyaeteritis nodosa affects which organ systems
skin
nerves
kidneys
MSK
GI
CNS
cardiac
polyarteritis nodosa lab results
Haematuria/proteinuria when renal involvement present
elevated CRP/ESR
anaemia, thrombocytosis
low albumin
hep b surface antigen
polyarteritis nodosa mild-moderate disease management
oral corticosteroids
polyarteritis nodosa life threatening disease management
high-dose pulsed IV corticosteroids
polyateritis nodosa additional therapy
Additionalimmunosuppressive therapyis usually required (e.g. azathioprine, methotrexate, cyclophosphamide or rituximab)
polyarteritis nodosa complication
aneurysm formation- lead to haemorrhage
Granulomatosis with polyangiitis (GPA) affects:
upper and lower respiratory tract, eyes and/or kidneys
typical age of GPA
35-50
ENT symptoms of GPA
sinusitis
nasal crushing
mouth ulcers
epistaxis (nose bleed)
sensourineal deafness
otitis media
‘saddle nose’
respiratory symptoms of GPA
cough
haemoptysis
pulmonary infiltrates
diffuse alveolar haemorrhage
cavitating nodules on CXR
occular symptoms of GPA
conjunctivitis, episcleritis, uveitis, optic nerve vasculitis, retinal artery occlusion, proptosis
cutaneous symptoms of GPA
palpable purpura, cutanous ulcers
Renal symptoms of GPA
necrotising glomerulonephritis
difference between GPA and EPGA
late onset asthma, high eosinophil count and ANCA specificity in EPGA
Microscopic polyangiitis very common clinical feature
Necrotising glomerulonephritis very common - occurs in up to 90% of patients
ANCA in GPA
cANCA
ANCA in EPGA and MPA
pANCA
most cases management of ANCA-associated small vessel vasculitis
IV steroids and cyclophosphamide
localised and systemic ANCA associated small vessel vasculitis management usually
methotrexate and steroids
generalised and systemic ANCA associated small vessel vasculitis management usually
Cyclophosphamide and steroids
renal biopsy of ANCA associated small vessel vasculitis
crescent formation
pauci-immune
HSP most common age range
children 2-11
HSP potential triggers
infection
medications
vaccines
insect bites
HSP symptoms
purpuric rash over buttocks and lower limbs
colicky abdominal pain
bloody diarrhoea
arthralgia/arthritis (knee and ankles predominantly)
HSP signs
fever
renal involvement
history of recent upper respiratory tract infection
HSP is a clinical diagnosis, what investigation is used for a definitive diagnosis in uncertain cases
tissue biopsy
Behçet’s disease most common in people from where
Turkey
Mediterranean
Japan
Behçet’s disease affects people with what HLA positivity
HLA-B51
Behçet’s disease symptoms
recurrent oral ulcers
recurrent genital ulceration
uveitis
erythema nodosum
test for Behect;s disease
the pathergy test- inflammatory papule or pustule, after mild skin trauma
polymyalgia rheumatica affects which age group
> 50 years
polymyalgia reumatica associated with
giant cell arteritis
polymyalgia reumatica symtoms
proximal myalgia of hip and shoulder girdles with morning stiffness lasting >45mins- usually symmetrical, occurs relatively quickly, pain worse with movement
systemic symptoms
polymyalgia reumatica signs
reduced movement of shoulders, neck, and hips
muscle strength normal
upper arm tenderness
carpel tunnel syndrome
pitting oedema
polymyalgia rheumatica management
rapid and dramatic response to low dose steroids- start at prednisolone 15mg daily
which ig mediates HSP
IgA
what is the most common vasculitis in children
HSP
