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MSK 2 > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis?

A

Inflammation in the blood vessel resulting in

  • Damaged blood vessels
  • Tissue ischaemia
  • aneurysms
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2
Q

What can cause secondary vasculitis?

A
  • Drugs
  • Other diseases (e.g. SLE, rheumatoid)
  • Malignancy
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3
Q

ANCA vasculitis

A

ANCA vasculitis, or antineutrophil cytoplasmic antibody-associated vasculitis, is the name of a group of autoimmune conditions characterized by the inflammation of the blood vessels caused by the immune system mistakenly attacking them.

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4
Q

Leukocytoclastic vasculitis

A

Leukocytoclastic vasculitis (LCV) refers to small blood vessel inflammation. It’s also known as hypersensitivity vasculitis and hypersensitivity angiitis. The word “leukocytoclastic” comes from leukocytoclasia, a process where neutrophils (immune cells) break down and release debris.

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5
Q

What is this called and what is it a sign of?

A

Palpable purpura - a sign of leukocytoclastic vasculitis.

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6
Q

What are the complications of vasculitis?

A

Stenosis - which can lead to tissue ischemia and necrosis.

Vessel wall injury which can lead to a weakened vessel wall and then aneurysm.

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7
Q

Large vessel vasculitis

A

The term ‘large vessel vasculitis’ encompasses the spectrum of primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches. The two major categories of large vessel vasculitis are temporal (giant cell) arteritis and Takayasu arteritis (TA).

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8
Q

What is the clinical presentation of giant cell arteritis?

A

Headache

  • scalp tenderness/ abnormal scalp vessels
  • occasionally ulceration

Pain on chewing

  • jaw pain
  • tongue pain

Visual disturbance/blindness

Unwell

Polymyalgia rheumatica

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9
Q

Why does giant cell arteritis present with jaw pain and pain when chewing?

A

GCA leads to the facial arteries that supply the masseter muscles becoming blocked and having a decreased blood supply, which leads to claudication in the masseter muscles.

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10
Q

Why does GCA lead to visual problems?

A

Inflammation to the blood vessels supplying the eyes can lead to the visual distrubance and blindness.

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11
Q

Which arteries are most affected in GCA?

A

Arteries most affected in giant cell arteritis are the temporal artery and other cranial arteries (now called cranial-GCA), but inflammation of the aorta and other large arteries in the body can occur as well and may present differently (now called large vessel-GCA).

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12
Q

Polymyalgia rheumatica

A

Polymyalgia rheumatica is a condition that causes pain, stiffness and inflammation in the muscles around the shoulders, neck and hips.

  • thought to be autoimmune
  • diagnosed based on bloods showing raised ESR and CRP.
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13
Q

What does this image show?

A

Temporal arteritis - GCA

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14
Q

What is GPA?

A

Granulomatosis with Polyangitis

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15
Q

How does GPA (granulomatosis with polyangitis) present?

A
  • destructive inflammation in nose / sinuses (saddle nose) / upper respiratory tract / eyes / mouth
  • lungs: cavitating lesions / coughing up blood due to pulmonary haemorrhage
  • kidneys: GN
  • arthritis / skin / nervous syndrome / heart / GI
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16
Q

What type of vasculitis is GPA?

A

PR3 c-ANCA

(Anti-neutrophil cytoplasmic antibodies)

17
Q

What is eosinophillic GPA?

A

Has the same presentation as GPA patients have a significant eosinophillia and asthma.

  • lungs and GI often involved.
  • aka Churg strauss
18
Q

What is MPO pANCA?

A

Microscopic polyangitis

19
Q

How does Microscopic polyangitis pANCA (Anti-neutrophil cytoplasmic antibodies) present?

A
  • fever, anorexia, weight loss
  • kidneys: GN
  • skin & wide-range of organ system small vessel inflammation / damage
20
Q

How does Behcet’s disease present?

A
21
Q

What is the treatment for GCA and PMR?

A

High dose corticosteroids for giant cell arteritis.

Low dose corticosteroids for polymyalgia rheumatica.

  • very good response - usually self limiting within 2 years.
22
Q

What is the main use for corticosteroids?

A

Corticosteroids are mainly used to reduce inflammation and suppress the immune system.

23
Q

Treatment for primary vasculitis:

A
  • glucocorticoids - used to dampen the immune response and inflammation.
  • rituximab (1st line in severe disease) - anti-B cell (CD20)
  • methotrexate (1st line in non-severe disease) or azathioprine
24
Q

How is Behcet’s treated?

A

Glucocorticoids - dampen down the immune response and inflammation.

Anti-TNF.

25
Q

Vasculitis overview:

A

Vasculitis is damage to the blood vessels due to inflammation which can lead to complications such as tissue ischemia or aneurysms.

Primary vasculitis is classified into the size of the vessels effected.

26
Q

Pathology of GCA:

A

Autoimmune inflammation of the carotid artery (especially the temporal branch of the carotid), opthalmic artery, facial arteries.

27
Q

Pathology of ANCA-associated vasculitis:

(Anti-neutrophil cytoplasmic antibodies)

A
  1. Antibodies bind to circulating neutrophils triggering a premature activation.
  2. The premature activation causes neutrophils to release reactive oxygen species along with pro-inflammatory cytokines and chemokines to attract more neutrophils.
28
Q

Pathology of Behcet’s vasculitis:

A

Behcet’s is an autoimmune disease is linked to HLA (human leukocyte antigen).

  • molecular mimicry (linked to excess production of H.pylori antibodies).
  • Linked to being Leukocytoclastic as leukocytes are found in vessel wall.
29
Q

Purpura presents in which type of vasculitis and why?

A

Small/medium vessel vasculitis as these vessels are more superficial and so the cutaneous manifestations such as purpura present here.

30
Q

Principle of autoimmunity in vasculitis:

A
  1. Molecular mimicry from pathogens can lead to CD8+ t cells responding to endothelial cells in the tunica intima or arteries instead of just the pathogens.
  2. Dendritic cells in the adventitia can become activated and release chemokines (chemical attractant for immune cells) - this attracts CD4 and CD8 lymphocytes mounting an immune response from the deep layer of the artery wall. This can result in chronic and granulomatis changes to the vessel wall seen in some forms of vasculitis.
  3. Antibodies produced by plasma cells can respond to endothelial cells in the artery lining due to molecular mimicry.
31
Q

Behcet’s disease affects which blood vessels?

A

Behcet’s affects all blood vessels - small, medium and large (both arterial venous).

32
Q

Why does tissue ischemia occur in vasculitis?

A
  1. Infiltration of immune cells leading to inflammation and destruction of the artery layers.
  2. Similar to atherosclerosis - inflammation to the vessel wall leads to damage which results in a platelet plug being formed and fibrin deposited - leading to stenosis of the artery.
  3. Hyperplasia, fibrosis and thickening of the artery wall.
  • all these mechanisms narrow the artery lumen leading to stenosis.
33
Q

Define aneurysm.

A

Dilation of a blood vessel due to an excessive localized swelling of the wall of an artery.

34
Q

ANCA vasculitis affects what size vessels?

A

Small vessels causing conditions such as granulomatosis polyangitis aka wergner’s.

35
Q

What is this deformity called and what vasculitis does it classicly present in?

A

Saddle nose deformity - classicly presents in c-ANCA - granulomatosis polyangitis.

36
Q

pANCA vs cANCA

A

pANCA = microscopic polyangitis

cANCA = granulomatosis with polyangitis

MSK 2 (20 decks)

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