Vasculitis Flashcards
What is vasculitis?
inflammation of the blood vessels
small vessels, medium sized vessels or 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
Generic presentation of most types of vasculitis (7)
Joint and muscle pain
Anterior uveitis and scleritis
Peripheral neuropathy
Gastrointestinal disturbance (diarrhoea, abdominal pain and bleeding)
Renal impairment
Hypertension
Purpura. These are purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin.
Systemic manifestations in vasculitis (5)
Fatigue
Fever
Weight loss
Anorexia (loss of appetite)
Anaemia
Tests in vasculitis
CRP and ESR
Anti neutrophil cytoplasmic antibodies (ANCA)
p-ANCA (MPO antibodies): Microscopic polyangiitis and Churg-Strauss syndrome
c-ANCA (PR3 antibodies): Wegener’s granulomatosis
Management of vasculitis
depends on the type
Steroids
Immunosuppressants
Different management in HSP and Kawasaki disease is different
What is HSP?
IgA vasculitis
Presents with a purpuric rash affecting the lower limbs or buttocks in children
What is a common trigger for HSP?
Upper airway infection
Gastroenteritis
At what age does HSP most commonly occur?
Children under the age of 10
Why does rash occur in HSP?
Inflammation occurs due to immunoglobulin A
Leaking of blood from small blood vessels under the skin
What are the 4 classic features of HSP?
Purpura (100%)
Joint pain (75%)
Abdominal pain (50%)
Renal involvement - IgA nephritis (50%)
Management of HSP
Typically supportive
Simple analgesia, rest and proper hydration
Recovery in HSP
Abdominal pain usually settles within a few days
Kidney involvement - full recovery in 4-6 weeks
1/3 have recurrence in 6 months
1% end stage renal failure
What is Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)?
Small and medium vessel vasculitis
Associated with lung and skin problems
What is the characteristic finding on FBC in Churg-Strauss?
Elevated eosinophil levels
How does Eosinophilic Granulomatosis with Polyangiitis often present?
Severe asthma in late teenage years or adulthood
What is Microscopic polyangiitis?
small vessel vasculitis
What are the features of Microscopic polyangiitis?
Main feature - renal failure
Can also affect lungs - SOB, haemoptysis
What is Granulomatosis with polyangiitis (Wegener’s granulomatosis)?
small vessel vasculitis
affects the respiratory tract and kidneys
What symptoms occur within the nose in Granulomatosis with polyangiitis?
Nosebleeds, crusty nasal secretions
Saddle shaped nose due to a perforated nasal septum - causes a dip halfway down the nose
What symptoms occur within the ears in Granulomatosis with polyangiitis?
Hearing loss
Tinitus
What symptoms occur in the lungs in Granulomatosis with polyangiitis?
Cough
Wheeze
Haemoptysis
What symptoms occur in the kidney in Granulomatosis with polyangiitis?
Rapidly progressing glomerulonephritis
What is Polyarteritis Nodosa?
Medium vessel vasculitis
Associated with hepatitis B
Can also occur in hep c or HIV
Where does polyarteritis nodosa affect?
Skin
Gastrointestinal tract
Kidneys
Heart
What rash is associated with polyarteritis nodosa?
Livedo reticularis
mottled, purplish, lace like rash
What is Kawasaki Disease?
Medium vessel vasculitis
Typically affects children under 5 years of age
Clinical features of Kawasaki disease
Persistent high fever > 5 days
Erythematous rash
Bilateral conjunctivitis
Erythema and desquamation (skin peeling) of palms and soles
“Strawberry tongue” (red tongue with prominent papillae)
What is a key complication of Kawasaki disease?
Coronary artery aneurysms
Treatment of Kawasaki
Aspirin
IV immunoglobulins
Takayasu’s arteritis (pulseless disease)
Large vessel vasculitis - can form aneuryms
Mainly affects the aorta
Also pulmonary arteries
When/how does Takayasu’s arteritis present?
Usually before the age of 40 years
Presents with:
Non-specific systemic symptoms - fever, malaise and muscle aches
or
Arm claudication or syncope
