Vasculitis Flashcards
what is vasculitis?
inflammation of blood vessels
lots of different types of vasculitis
small vessels
medium sized vessels
large vessels
types of vasculitis affecting the small vessels
Henoch-Schonlein purpura
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Microscopic polyangiitis
Granulomatosis with polyangiitis (Wegener’s granulomatosis)
types of vasculitis affecting the medium sized vessels
Polyarteritis nodosa
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Kawasaki Disease
types of vasculitis affecting the large vessels
Giant cell arteritis
Takayasu’s arteritis
how might vasculitis present?
Purpura. These are purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin.
Joint and muscle pain
Peripheral neuropathy
Renal impairment
Gastrointestinal disturbance (diarrhoea, abdominal pain and bleeding)
Anterior uveitis and scleritis
Hypertension
*fatigue, fever, weight loss, anorexia, anaemia
what are the tests for vasculitis?
bloods:
CRP, ESR
special:
ANCA (anti neutrophil cytoplasmic antibodies)
pANCA (anti MPO) (microscopic polyangitis, churg-strauss syndrome)
cANCA (anti PR3) (weighers granulomatosis)
management of vasculitis
depends of the type
referral to rheumatology
medical:
- steroids (oral: predniosolone, IV: hydrocortisone, nasal, inhaled)
- immunosupressants cyclophosphamide methotrexate azathioprine rituximab
HSP
IgA vasculitis
purpuric rash which affects lower limb and buttocks in children
deposition of IgA in blood vessels
affects skin, kidneys, GI tract and often triggered by an URTI (tonsillitis) gastroenteritis
the rash is due to leaking of blood from small vessels under the skin (purport)
- purpura
- joint pain
- abdo pain
- renal involvement (IgA nephritis)
management:
supportive, analgesia, rest and hydration, steroids
eosinophilic granulomatoiss with polyangitis (churg strauss syndrome)
small and medium vessel vasculitis
lung and skin
can affect other organs
severe asthma
! raised eosinophils on FBC
microscopic polyangitis
small vessel vasculitis
renal failure
can affect lung causing SOB and haemoptysis
Wegner’s granulomatosis
granulomatosis with polyangitis
small vessel vasculitis
affects resp tract and kidneys
nose (epistaxis) and crusty nasal secretions
ears - hearing loss
sinusitis
saddle shaped nose due to perforated nasal septum
lung: wheeze, cough, haemoptysis (X-ray shows condoliation)
kidney - glomeulonephrits
polyarteritis nodosa
medium vessel vasculitis
hep B, hep C, HIV
effects skin, GI tract, kidney and heart.
renal impairment, strokes, MI
livedo reticularis
mottled, purplish, lace like rash
Kawasaki disease
medium vessel vasculitis
young children <5 y/o
high fever >5 days erythematous rash bilateral conjunctivitis erythema desquamation of palms and soles (skin peeling) strawberry tongue
complication is coronary artery aneurysm. treat with aspirin and IV immunoglobulins
takaysau’s arteritis
large vessel vasculitis
affects aorta and branches
pulmonary arteries
40 y/o
non specific: fever, malaise, muscle aches, arm claudication, syncope
investigate: CT, MRI, doppler US of carotid arteries.
GCA- giant cell arteritis
systemic vasculitis of medium and large arteries
typically affects temporal arteries (temporal arteritis)
symptoms of GCA
- Severe unilateral headache typically around temple and forehead
- Scalp tenderness my be noticed when brushing hair
- Jaw claudication
- Blurred or double vision
- Irreversible painless complete sight loss can occur rapidly
- Fever
- Muscle aches
- Fatigue
- Weight loss
- Loss of appetite
- Peripheral oedema
complication- vision loss
link with polymyalgia rheumatic
how is GCA diagnosed?
clinical presentation
bloods: raised ESR >50mm/hour FBC: normocytic anaemia, thrombocytosis LFT raised ALP CRP
imaging:
duplex ultrasound of temporal artery (hypo echoic halo sign)
special:
temporal artery biopsy
shows multinucleate giant cells
management of GCA
conservative
medical:
steroids: 40-60mg prednisone (60mg if jaw claudication or visual symptoms) review in 48 hours
aspirin 75mg
PPI (while on steroids)
referral:
vascular surgeon
rheumatology
opthalmology
steroids ongoing DONT STOP
DON’T – Don’t stop taking steroids abruptly. There is a risk of adrenal crisis.
S – Sick Day Rules.
T – Treatment Card.
O – Osteoporosis prevention with bisphosphonates and supplemental calcium and vitamin D.
P – Proton pump inhibitor for gastric protection.
complications of GCA
Early neuro-ophthalmic complications:
Vision loss
Cerebrovascular accident (stroke)
Late:
Relapses of the condition are common
Steroid related side effects and complications
Cerebrovascular accident (stroke)
Aortitis leading to aortic aneurysm and aortic dissection
antiphospholipid syndrome
disorder associated with antiphospholipid antibodies. blood becomes prone to clotting (hyper-coagulable state)
- thormobisis
- pregnancy- recurrent miscarriage
can exist alone or secondary to SLE
antiphospholipid antibodies
Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies
they interfere with coagulation
associated symptoms of antiphospholipid syndrome
venous thromboembolism
DVT, PE
arterial thrombosis:
stroke, MI, renal thrombosis
pregnancy:
recurrent miscarriage
still birth
pre eclampsia
livedo reticularis
libmann-scks endocarditis (mitral valve)
thrombocytopenia
how is antiphospholipid syndrome diagnosed?
history of thrombosis / pregnancy complications
Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies
management of antiphospholipid syndroem
rheumatology
haematology
obstetrics (if pregnant)
medical:
warfarin INR 2-3
(3-4 if recurrent thrombosis)
LMWH (enoxaparin)
aspirin
Behcet’s disease
a complex inflammatory condition. oral and genital ulcers. inflammation of skin, GI, lung, blood vessels, MSK and CNS
link with HLA-B51 gene
differential diagnosis of Behcet’s disease
(mouth ulcers)
Simple aphthous ulcers are very common
Inflammatory bowel disease (particularly Crohn’s disease)
Coeliac disease
Vitamin deficiency (B12, folate or iron)
Herpes simplex ulcers
Hand, foot and mouth disease (coxsackie A virus)
Squamous cell carcinoma
features of Behcets disease
mouth ulcers- 3 episodes per year. painful, sharply circumscribed erosions, red halo. heals 2-4 weeks
genial ulcers- kissing ulcers (two opposing surfaces facing each other)
skin- erythema nodosum, papule and pustules, vasculitis type rashes
eyes- anterior/posterior uveitis, retinal vasculitis, retina haemorrhage
MSK - morning stiffness, arthralgia, oligoarthritis
GI- ileum, cecum ascending colon inflammation and ulceration
CNS- memory impairment, headaches, migraine, aseptic menigntisi, meningoencephalitis
veins- buddd chiari, DVT, thrombus in pulmonary veins, cerebral venous sinus thrombosis
lungs- pulmonary artery aneurysm
investigation for Behcet’s disease
pathergy test: sterile needle to create a subcutaneous abrasion on the forearm. reviewed 24-48 hours later to look for a weal of 5mm or more. +ve
management of Behcet’s disease
rheumatology
opthalmology
dermatology
neurology
conservative
medical: steroids- soluble bethamethasone for mouth ulcers systemic steroids- prednsiolone colchine - anti inflamamotry topical anaesthetic- lidocaine immunosupressants- azathoprine biological therapy infliximab
prognosis for Behcet’s disease
relapsing remitting
increased mortality with haemoptysis, neurological involvement
