Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

MsK > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis

A

Inflammation of a blood vessel (arteries or veins), which is characterised by the presence of an inflammatory infiltrate and destruction of the vessel wall.
Heterogenous conditions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What does endothelial injury lead to

A

Results in thrombosis + ischaemia/infarction of dependent tissues

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Types of Vasculitis Affecting The Large Vessels: arteries and major tributaries

A
  • Giant cell arteritis
  • Takayasu’s arteritis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Types of Vasculitis Affecting The Medium Sized Vessels: small arteries and arterioles

A
  • Polyarteritis nodosa
  • Kawasaki Disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Types of Vasculitis Affecting The Small Vessels: small arteries, arterioles, venuoles and capillaries

ANCA +ve:

A
  • Microscopic polyangiitis
  • Granulomatosiswith polyangiitis (Wegener’s granulomatosis)
  • Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Types of Vasculitis Affecting The Small Vessels: small arteries, arterioles, venuoles and capillaries

ANCA -ve:

A

Henoch-Schonlein purpura
Cryoglobulinemic vasculitis
Anti-C1q vasculitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Aetiology of vasculitis

A

May be primary (this may be due to direct or indirect damage of endothelial cells of the vessel) or secondary to other conditions such as RA, SLE, hepatitis B & C, HIV, polymyositis and some allergic drug reactions.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is medium and large vessel vasculitis typically due to?

A

due to an autoimmune disease, where the immune system confuses a part of normal body as a foreign invader

body confuses the innermost layer of the blood vessel, which is the endothelial layer, with a foreign pathogen and directly attacks it

white blood cellsmix up the normal antigens on the endothelial cells with the antigens of foreign invaders like bacteria -

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what are small vessel vasculitides due to?

A

immune system attacks healthy cells that are near the vascular endothelium, and the endothelial cells are only getting indirectly damaged.

situation in many small-vessel vasculitides, where theimmune system attacks white blood cell enzymes or other non-endothelial cell targets.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

How do all vasculitis progress basically?

A

damaged endothelium exposes the underlying collagen and tissue factor, and these exposed materials increase the chance of blood coagulation
Blood vessels - weaker and become damaged - more likely aneurysms - as vessel wall heals becomes harder and stiffer as fibrin deposited in vessel wall as part of healing proces

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What does presentation of vasculitis depend on?

A

vessel affected and the corresponding organ - could have ischaemia

  • Blood cells clump onto the exposed tissue factor and collagen on the inside of blood vessels forming blood clots that can restrict blood flow.
  • ## As fibrin is deposited in the vessel wall, the walls become thicker and bulge into the vessel, reducing the diameter of the vessel lumen, and restricting blood flow.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Typical general symptoms of vasculitis

A
  • Fatigue
  • Fever
  • Weight loss
  • Anorexia (loss of appetite)
  • Anaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Typical specific presentations (depending on vessel and organ affected)

A
  • Joint and muscle pain
  • Peripheral neuropathy - vessels to the head are affected
  • Anterior uveitis and scleritis - vessels to the head affected, causing ischaemia of the eye
  • 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.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Markers for vasculitis

A
  • Elevated ESR and CRP
  • Anti neutrophil cytoplasmic antibodies (ANCA) - positive in most small vessel vasculitis
    • 2 types: P-ANCA are also called anti-MPO antibodies. C-ANCA are also called anti-PR3 antibodies.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

General management for vasculitis

A

Steroid:
Prednisolone - oral
Hydrocortisone - IV
Nasal sprays

Immunosuppressants:
- Cyclophosphamide
- Methotrexate
- Azathioprine
- Rituximab and other monoclonal antibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is Giant cell arteritis (GCA)

A

Inflammation of the lining of your arteries

Sometimes called temporal arteritis as it commonly affects the temporal branch of the carotid artery.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What does giant cell arteritis co exist with normally?

A

Polymyalgia Rheumatica (PMR)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Epidemiology of GCA

A
  • Common in elderly >55 years.
  • F>M
  • Associated with PMR in 50%
    -0.4% of people
  • commonest vasculitis
  • common in white people
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Pathophysiology of GCA

A
  • Arteries become inflamed, thickened and can obstruct blood flow
  • Cerebral arteries affected in particular e.g. temporal artery
  • Opthalmic artery can also be affected potentially resulting in permanent or
    temporary vision loss
  • Blue dots around adventitia media and intima, cells blur margin between media and adventitia - intima proliferated into lumen causing it to narrow
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

S + S of GCA (non cranial)

A
  • Malaise
  • Dyspnoea
  • Weight loss
  • Morning stiffness
  • Unequal or weak pulse
  • anaemia
  • limb claudication
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Symptoms of GCA due to arteries in the head being affected (Cranial GCA)

A
  • New Headache
  • Scalp tenderness (pain when combing hair)
  • Tongue/ jaw claudication - pain when chewing - lingual or facial artery involvement
  • Amaurosis fugax - unable to see from one or both eyes
  • Sudden unilateral blindness
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Bloods in GCA

A
  • Elevated ESR and CRP
  • Elevated ALP
  • Elevated platelets
  • Reduced Hb
  • ANCA -ve
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Biopsy in GCA (temporal artery)

A
  • Can see skip lesions - GCA is segmental so doesn’t affect the whole length of the artery
  • Histology - giant cells (granulomas) seen in the internal elastic lamina
    Specifity 100%, sensitivity 39%
24
Q

Diagnostic criteria for GCA

A
  • Age at disease onset ≥50 years
  • New headache
  • Temporal artery abnormality
  • Elevated ESR(> 50 mm/hr)
  • Abnormal temporal artery biopsy(typical vasculitis features)

3 of the following criteria have to be met

25
Q

Treatment for GCA

A

Steroids - dampen down immune response - no delay in corticosteroid treatment
- Prednisolone
- IV methylprednisolone if evolving visual loss
Long term steroids need GI and bone protection:

  • PPI
  • Ca2+ and Vit D
  • Biphosphonate
26
Q

Prognosis for GCA

A

Typically a 2 year course and then complete remission. Can reduce prednisolone once symptoms have resolved. Symptoms may reoccur.

Main cause of death is long term steroid treatment!

27
Q

What is Granulomatosiswith polyangiitis (Wegener’s granulomatosis) WG

A

multi-system disorder of unknown causes characterised by necrotising granulomatous inflammation and vasculitis of small and medium vessels.

28
Q

Epidemiology of WG

A

Usually occurs in middle-aged men

29
Q

What are cANCAs

A

B-cells target antibodies to granules made by a person’s neutrophils. The antibodies are called ‘cytoplasmic anti-neutrophilic cytoplasmic antibodies’ or cANCAs.

30
Q

Pathophysiology of WG

A

c-ANCA targets and bind to a specific neutrophil granule called proteinase 3 which is embedded in the membrane of some neutrophils.

Once c-ANCA binds to the neutrophil, it causes the neutrophil to release oxygen free radicals, which can then indirectly damage the nearby endothelial cells, causing vasculitis.

31
Q

Upper respiratory tract issues in WG

A
  • Chronic pain caused by sinusitis
  • Bloody mucus due to ulcers within the nose
  • Saddle nose deformity: nose caves in
  • Hearing loss
32
Q

Lung issues in WG

A
  • Breathing difficulty due to inflammation
  • Wheeze
  • **Ulcers can cause bloody coughing*
33
Q

Kidney issues in WG

A
  • Decreased urine production due to glomeruli death**
  • Proteinuria**
  • Haematuria**
  • Increase in BP
34
Q

Investigations for WG

A

FBC:
- cANCA +ve
- ESR and CRP raised

  • Urinalysis - to look for proteinuria and haematuria
  • Renal biopsy - can see granulomas and inflammation in blood vessel wall
  • Chest x-ray - may show nodules +/- fluffy infiltrates of pulmonary haemorrhage
  • CT - may reveal diffuse alveolar haemorrhage
35
Q

Management of WG

A
  • Steroids combined with cyclophosphamide
  • Methotrexate and azathioprine usually used for maintenance
  • Plasma exchange in patients with severe renal disease or pulmonary haemorrhage
36
Q

What is Polyarteritis nodosa? PN

A

Necrotising vasculitis - causes aneurysms and thrombosis in medium sized arteries, leading to infarction in affected organs with severe systemic symptoms.

37
Q

Epidemiology of PN

A
  • M>F
  • Rare in the UK
  • May be associated to hepatitis B
38
Q

Pathophysiology of PN

A

occur when the immune cells directly attack the endothelium, confusing it with hepatitis B virus.

Causes transmural inflammation - causes vascular wall to die through all 3 layers of artery - fibrosis occurs as vascular wall heals - process called fibrinoid necrosis

Fibrosed vessel wall left weak and prone to aneurysms

39
Q

What is transmural inflammation

A

means the entire wall, the tunica intima, the media, and the adventitia are all affected.

40
Q

General symptoms of PN

A

Fever, malaise, weight loss and myalgia

41
Q

What happens if renal arteries affected in PN

A
  • Hypertension
  • Haematuria
  • Proteinuria
42
Q

What happens if mesenteric artery is affected in PN

A

Abdominal bleeding
GI bleeding

43
Q

What happens if arteries supplying the brain are affected in PN

A

Neurological symptoms e.g. numbness, tingling, abnormal/lack of sensation and inability to move part of the body

44
Q

What happens if arteries supplying the skin are affected?

A
  • Skin lesions
  • Rash - Livedo reticularis (pink blue mottling caused by stasis of blood in skin venules)
  • Punched-out ulcers
  • Nodules
45
Q

Cardiac issues in PN

A

Coronary arteritis causes myocardial infarction and heart failure

46
Q

Investigation for PN

A

FBC:
- Elevated WCC
- Eosinophilia
- Anaemia
- Raised ESR and CRP
- ANCA -ve

  • Renal or mesenteric angiogram - can see string of beads pattern due to fibrotic aneurysms
  • Renal biopsy
47
Q

Management of PN

A
  • Steroids e.g. prednisolone in combination with immunosuppressive drugs e.g. azathioprine or cyclophosphamide
  • Control BP
  • Hep B should be treated after treatment with steroids
48
Q

Healthy vessel

A

Out to in
Adventitia - has vasa vasorum
Media
Intima

49
Q

General pathophysiology of vasculitis

A

Primary phenomena = idopathic immune process
Secondary phenomena = drugs, RA, infection
Activated immune cell infiltrate vessel wall, direct damage and stimulates vascular smooth muscle remodelling
Infiltration, proliferation and damage causes weaking and occlusion of blood vessel

50
Q

Complications for GCA

A

Strokes
Blindness - stroke affecting retinal vessels in optic nerve
Bilateral simultaneous or sequential loss

51
Q

What other scans should be given for confirmatory test

A

Temporal artery US
Temporal artery biopsy
or both
PET- CT + Auxillary US to look for extra cranial disease

52
Q

Managing steroid complication and pre existing co-morbidities

A

Steroid induced diabetes
Osteoporosis
Infection
Gi toxicity

53
Q

Where are smooth muscle cells found

A

Tunica Media

54
Q

Two forms of classificiation of vasculitis

A

1- by vessel size
2- consensus classification

55
Q

Two most common clinical presentations of GCA

A

Cranial GCA
Large Vessel GCA

56
Q

Physical signs of GCA

A

-Scalp tenderness
- temporal artery

MsK (14 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions