Vasculitis

Question 1

Vasculitis definition

Answer 1

A group of conditions in which inflammation and damage to blood vessels is the primary underlying pathology

Question 2

ANCA associated vasculitis

Answer 2

In ANCA-associated vasculitis, ANCA specifically bind to two proteins that are normally found in the fluid within the neutrophil (cytoplasm).

Question 3

Eisonophilic granulomatosis with polyangitis (Churg-Strauss syndrome)

Answer 3

Small vessel vasculitis

ANCA position (30-50% ANCA-positive)
– (antineutrophylic cytoplasmic antibody)
– ANCA’s target neutrophils that are involved with the immune response

Has a large lung and skin involvement

Blood eosinophilia and previous history of asthma

Mean age of 40, most patients who suffer with this are adults

Symptoms:
– rash
– peripheral neuropathy
– eosinophilia in the blood
– glomerulonephritis
– late onset asthma

Question 4

Rheumatoid vasculitis

Answer 4

A rare but very serious complication of rheumatoid arthritis (RA)- due to RA being able to speed through the blood, sometimes it can affect the blood vessels (known as rheumatoid vasculitis)

Usually causes inflammation to the small/medium sized vessels, and most commonly affects the skin, nerves, fingers and toes

Main symptoms:
– skin rash
– small pits on fingertips
– redness around nails
– numbness, tingling , and pain in the hands and feet

Question 5

Buerger’s disease

(Thromboangiitis obliterans)

Answer 5

Rare disease of the arteries and veins in the arms and legs.
– affects the small vessels of the lower limb

Blood vessels can become blocked- restring the blood flow to the affected areas. Blood clots may form in the blood vessels

Over time, the lack of blood flow damages or destroys the skin tissue
– this damage can lead to infection and death of body tissue, called gangrene.

Symptoms:
– tingling or numbness in the fingers or toes
– changes in skin colour in the hands and feet
– skin may be pale gray, red or blue
– fingers and toes that turn white then blue when exposed to cold, known as Raynaud’s disease.
– pain in the feet that occurs with walking

Question 6

Bachets disease

Answer 6

Rare disorder that causes blood vessel inflammation throughout your body

Unknown cause

Signs and symptoms:
– mouth sores
– eye inflammation
– skin rashes and lesions
– genital sores

—- these may be seen as first signs that seem useless but are telling signs

Symptoms:
– mouth- sores on moth
– skin- acne-like sores
– genitals- open sores on scrotum
– eyes- inflammation in the eye (uvetis)
– joints- swelling and pain often affect the knees

Question 7

Kawasaki’s disease

Answer 7

Acute systemic vasculitis involving medium sized vessels

Ep:
– affects mainly children under 5 years of age

Very frequent in Japan

An infective trigger is suspected

CP:
– fever lasting 5 days or more
– conjunctivitis
– coronary arteritis
– dryness and redness of the lips and oral cavity 3 days after onset
– acute cervical lymphadenopathy accompanying the fever
– polymorphic rash involving any part of the body
– redness and oedema of the palms and soles 2-5 days after onset

Cardiovascular changes in the acute stage- pancarditis and coronary arteritis leading to aneurysms or dilatation

Treatment:
– single dose of high-dose IV immunoglobulin- prevents coronary artery disease

Question 8

Granulomatosis

Answer 8

Granulomatosis with polyangiitis (Wegener’s granulomatosis)

A systemic ANCA-associated vasculitis characterised by dominant upper respiratory tract, lung and real involvement
cANCA positivity

CP:
– nasal symptoms
– acute renal failure
– pulmonary symptoms
– focal necrotising segmental necrotising glomerulonephritis with crescent formation
– large geographical areas of necrotizing granulomatous inflammation and a necrotising vasculitis

Aggressive immunosuppression is needed to prevent mortality