Vasculitis Disorder

Question 1

Vasculutis definition

Answer 1

Inflammation in the vessel walls

Question 2

ANCAs and what levels of ANCAs signfiy

Answer 2

Antineutrophil Cytoplasmic Antibodies, one of the reasons for vasculitis. There are antibodies against the granules of the neutrophils. There are types of ANCAs:

1. MPO-ANCAs: anti myeloperoxidase ANCAs
2. Anti peroxidase 3 ANCAs - associated with Wenger Granulomatosis disease, this is a type of vasculitis

Titers of ANCAs can be monitored to assess the effectiveness of the treatment

Question 3

Describe the pathological changes and clinical features of giant cell arteritis

Answer 3

It is a form of T cell mediated autoimmune diseasem, type IV, caysed by TNFalpha

Question 4

Answer 4

Giant cell arteritis, blood flow is completely obstructued and hence there is blindness since there is no blood flow to the eye

Question 5

Answer 5

Giant cell arteritis

Question 6

Answer 6

Fragmentation of elastic lamina associated with giant cell arteritis

Question 7

Takayasu ateritis

Answer 7

Happens mainly in the aorta and its branches. It is associated with thickening of the vessel wall and narrowing of the lumen of the vessel.

Symptoms are high blood pressure, assymetirc blood pressure with weaker pulses in different parts of the body, there can be occular distrubrance and sight changes and there are can be cardiac symptoms

Question 8

Answer 8

Intima is markedly markedly thick, this is Takayasu arteritis

Question 9

What are these pictures showing

Answer 9

Tunica media is completely normal whereas the intima is markedly thick, this is takayasu arteritis

Question 10

Answer 10

Granulomatous inflammation seen in the aorta as part of the Takayasu arteritis

Question 11

Polyarteritis Nodosa

Answer 11

Causes fibrinoid necrosis in medium to small sized vains.

Question 12

Answer 12

Polyarteritis nodosa

Question 13

Answer 13

Polyarteritis Nodosa, fibrinoid necrosis

Question 14

Answer 14

Strong evidence of fibrinoid necrosis, polyarteritis nodosa

Question 15

Kawasaki disease

Answer 15

Acute necrotizing vasculitis of infance and early childhood with fever and rash

It is also caused by mucocutanrous lymph node syndrome

Question 16

Wenger Granulomatous Disease

Answer 16

Affects small vessels in an aggressive way, it is classified as systemic necrotizing granlumatous vasculitis of unknown etiology.

Clinical features include: hematouria and protinurea, usually affects the lungs, similar to goodpasteur syndrome, affects the lungs by pneumonitis

Question 17

Answer 17

Wegner disease

Question 18

Churg-Strauss Syndrome

Answer 18

Systemic vasculitis with prominent eosinophilia that occurs in young people with asthma or allergy. There is necrotizing vasculitis in the smal and medium sized vessels, there are granulomas, eosinophilia and fibrinoid necrosis so its similar to polyarteritis nodosa. Can have a range of clinical symptoms depending on what organ is infected since this is a systemic disease

Question 19

Hypersensitivity Angiitis or leukocytoclastic vasculitis or micoscopic polyangiitis

Answer 19

A group of inflammatory vascular lesions affecting small vessels that are thought to represent exogenous substances.

It is often drug induced or can be due to bacteria.

Question 20

Answer 20

Purpura - palpable cutaneous hemorrhages, this is hypersensitivity angiitis

Question 21

Answer 21

Hypersx Angiits

Question 22

Answer 22

Hypersx Angiitis

Question 23

Thromboangiitis Obliterans

Answer 23

Occlusive inflammatory disease of the medium and small arteries in the arms and legs

Happens most often in smokers and middle aged men

There is narrowing of the vessel wall by acute inflammation which can lead to thrombosis and infarcts. It can causes gangerenes to develop in the periphery and it is also associated with Raynaud’s phenomena

Question 24

Answer 24

Thromboangiitis Obliteran