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Vasculitis Flashcards

1
Q

What is vasculitis?

A

Vasculitis is inflammation of the blood vessel, often leading to ischaemia and necrosis of the tissue

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2
Q

What are the 3 main sub-types of vasculitis?

A

Large vessel
Medium vessel
Small vessel

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3
Q

What are the 3 sub-groups of small-vessel vasculitis?

A

Immune complex small-vessel vasculitis
Anti-GBM disease
ANCA-associated small-vessel vasculitis

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4
Q

What are some examples of large-vessel vasculitides?

A

Takayasu’s arteritis
Giant cell arteritis

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5
Q

What are some examples of medium-vessel vasculitides?

A

Polyarteritis nodosa
Kawasaki disease

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6
Q

What are some examples of immune complex small-vessel vasculitides?

A

Henoch-Schönlein
Hypocomplementemic urticarial vasculitis (Anti-C1q vasculitis)

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7
Q

What are some examples of ANCA-associated small-vessel vasculitides?

A

Microscopic polyangiitis
Granulomatosis with polyangiitis (Wegener)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

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8
Q

What are some factors that can cause secondary vasculitis?

A

Infection
Drugs
Toxins
Other inflammatory disorders
Cancer

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9
Q

What are some common features of all vasculitides?

A

Fever
Malaise
Weight loss
Fatigue

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10
Q

What is Takayasu’s arteritis?

A

This is a large cell vasculitis, most commonly affecting the aortic arch

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11
Q

Who is most at risk of Takayasu’s arteritis?

A

This is most commonly found in those under 40 years and in females

It is also more prevalent in Asian populations

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12
Q

What are some non-conventional features of Takayasu’s arteritis?

A

Claudication in arms
Blood pressure differences in extremities
Bruit (Most commonly in carotids)

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13
Q

What investigations are required in Takayasu’s arteritis?

A

Blood tests - Raised inflammatory markers
CT or MR angiography - Stenosis and vessel wall thickening

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14
Q

How is Takayasu’s arteritis usually managed?

A

Initial management of large vessel vasculitis is 40-60mg prednisolone, with steroid sparing agents given in Takayasu’s arteritis

These include leflunamide and methotrexate

Tocilizumab is a biologic that can be given

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15
Q

What is another name given to giant cell arteritis?

A

temporal arteritis

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16
Q

What is giant cell arteritis?

A

A type of systemic vasculitis affecting the large and medium arteries

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17
Q

Who is most at risk of Giant cell arteritis?

A

It is more commonly found in those over 50, and has a strong link with polymyalgia rheumatica and white ethnicity

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18
Q

What are some un-conventional symptoms of giant cell arteritis?

A
  • Unilateral acute temporal headache (Temporal artery)
  • Scalp tenderness
  • Temporary visual disturbances (Ophthalmic artery)
  • Blindness (Ophthalmic artery)
  • Jaw claudication
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19
Q

What are some features of giant cell arteritis not affecting the head?

A

Carpel tunnel syndrome
Peripheral oedema

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20
Q

What is the main clinical sign of giant cell arteritis?

A

Inflamed temporal artery

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21
Q

What investigations are required in giant cell arteritis?

A

Bloods - Raised ESR, CRP
USS
Temporal artery biopsy
PET, CT or CT angiogram

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22
Q

Why might a temporal artery biopsy be negative in giant cell arteritis?

A

Giant cell arteritis causes skip lesions

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23
Q

How is giant cell arteritis managed?

A

Initial management of large vessel vasculitis is 40-60mg prednisolone, with steroid sparing agents considered in GCA
These include leflunamide and methotrexate
This dose is then reduced over 18 -24 months

Tocilizumab is a biologic that can be given

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24
Q

What medication is given in giant cell arteritis, in cases of jaw claudication or visual symptoms?

A

In cases of visual symptoms or jaw claudication, 500-1000mg of methylprednisolone is given daily

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25
What drugs can be given to counteract steroid side effects in giant cell arteritis?
PPIs, bisphosphonates, calcium and vitamin D can be given to counteract side effects of steroids (Gastric ulceration and bone destruction)
26
What is polyarteritis nodosa?
This is a systemic medium vessel condition occurring mostly in middle age to older patients
27
What are some causes of secondary polyarteritis nodosa?
Hepatitis B Hepatitis C
28
What are some renal symptoms of polyarteritis nodosa?
Renal hypertension Renal function impairment
29
What are some cutaneous presentations of polyarteritis nodosa?
Purpura Livido reticularis Ulcers
30
What is a common musculoskeletal symptoms of polyarteritis nodosa?
Muscle pain
31
What are some neurological presentations of polyarteritis nodosa?
Mononeuritis Polyneuropathy
32
What are some gastrointestinal presentations of polyarteritis nodosa?
Abdominal pain Diarrhoea (±blood) Bowel perforation
33
What is livido reticularis?
A transient or persistent, blotchy, reddish-blue to purple, net-like cyanotic pattern that occurs in polyarteritis nodosa
34
What investigations are required in polyarteritis nodosa?
Biopsy of affected tissue Angiogram There are no specific blood tests
35
How is non-organ threatening polyarteritis nodosa treated?
In cases of non-organ threatening disease steroids are given with an immunosuppressant, such as azathioprine, methotrexate or mycophenolate
36
How is organ threatening polyarterits nodosa treated?
Steroids are given with cyclophosphamide
37
What is Kawasaki disease?
This is a very rare, medium cell vasculitis, also known as mucocutaneous lymph node syndrome, occurring mostly in children under 5
38
Who is most at risk of Kawasaki disease?
Children under 5 Children from Northeastern Asia, especially Japan and Korea
39
How does Kawasaki disease present?
Initially it presents as a normal infection, however, it then goes on to cause symptoms such as: - High fever - Widespread rash - Bilateral conjunctivitis - Strawberry tongue - Coronary artery aneurysm
40
What are the 2 main treatments of Kawasaki disease?
Aspirin IV immunoglobulin
41
What is used in Kawasaki disease that does not respond to IV immunoglobulin therapy?
Corticosteroids
42
What are some general MSK and cutaneous symptoms of small vessel vasculitides?
Palpable purpura Cutaneous ulcers Arthrlagia Myalgia/Myositis
43
What are some common ENT presentations in small vessel vasculitides?
Sinusitis Nasal crusting Epistaxes Mouth ulceration Otitis media and deafness Saddle nose (Cartilage damage) Sub-glottic inflammation
44
What are some respiratory symptoms of small vessel vasculitides?
Cough haemoptysis Pulmonary infiltrates Diffuse alveolar haemorrhage Cavitating nodules Asthma
45
What are some ocular symptoms of small vessel vasculitides?
Conjunctivitis Episcleritis Uveitis Optic nerve artery vasculitis Retinal artery occlusion Proptosis-pseudotumour
46
What are some neurological symptoms of small vessel vasculitides?
mononeuritis multiplex Sensorimotor polyneuropathy Cranial nerve palsy
47
What is ANCA?
Anti-Neutrophil Cytoplasmic Antibodies A form of auto-antibodies against the cytoplasms of neutrophils within the blood vessels
48
What are the 2 forms of ANCA antibodies?
P-ANCA - MPO antibody - Against nucleus cytoplasm (P = Perinuclear) C-ANCA - PR2 antibody - Against all cytoplasm (C= Cytoplasmic)
49
What is Granulomatosis with polyangiitis (Wegener's)
This is a vasculitis characterised by granulomatous inflammation, affecting the small and medium sized vessels of the lungs, eyes or kidneys
50
Who is most at risk of developing GPA?
Northern Europeans Males (Slightly) 35-55 years old
51
What types of symptoms will occur in GPA?
Respiratory symptoms Necrotising glomerulonephritis Granulomatous inflammation Nasal symptoms Ocular features
52
What respiratory symptoms are most common in GPA?
Upper airways disease Pulmonary parenchymal disease
53
What investigations are required in small vessel vasculitides?
Bloods urine dipstick testing Immunofluorescence Biopsy
54
What will blood testing show in small vessel vasculitides?
Raised ESR, PV and CRP Anaemia in chronic disease Complement activation Abnormal U+E in renal involvement
55
What will be seen on urine dipstick testing in small vessel vasculitides?
Haematuria
56
What is the predominant antibody in GPA?
P-ANCA
57
What further tests can be performed in small vessel vasculitis to show organ involvement?
CT chest scan Nerve conduction testing Muscle MRI Tissue biopsy
58
How is remission induced in organ threatening GPA?
- Glucocorticoids + Rituximab/Cyclophosphamide - Avocopan - C5 inhibitor may be added - Plasma exchange when creatinine >300umol
59
How is remission maintained in organ threatening GPA?
- Tituximab or azathioprine/methotrexate - Mycophenolate or leflunamide
60
How is resistant disease managed in organ threatening GPA?
- Rituximab + Cyclophosphamide - IV immunoglobulin therapy
61
How is remission induced in non-organ threatening GPA?
Glucocorticoids + Rituximab/Methotrexate/Mycophenolate
62
How is remission maintained in non-organ threatening GPA?
- Rituximab/Azathioprine/Methotrexate - Mycophenolate or Leflunamide
63
What is microscopic polyangiitis?
Microscopic polyangiitis is a necrotising vasculitis of the small vessels, with few immune deposits, typically with pulmonary, renal and skin involvement
64
What are the most common presentations of microscopic polyangiitis?
Glomerulonephritis Nervous system dysfunction Diffuse alveolar haemorrhage
65
What is the predominant antibody found in microscopic polyangiitis?
C-ANCA
66
How is remission induced in organ threatening microscopic polyangiitis?
- Glucocorticoids + Rituximab/Cyclophosphamide - Avocopan - C5 inhibitor may be added - Plasma exchange when creatinine >300umol
67
How is remission maintained in organ threatening microscopic polyangiitis?
- Tituximab or azathioprine/methotrexate - Mycophenolate or leflunamide
68
How is resistant organ threatening microscopic polyangiitis managed?
- Rituximab + Cyclophosphamide - IV immunoglobulin therapy
69
How is remission induced in non-organ threatening microscopic polyangiitis?
Glucocorticoids + Rituximab/Methotrexate/Mycophenolate
70
How is remission maintained in non-organ threatening microscopic polyangiitis?
- Rituximab/Azathioprine/Methotrexate - Mycophenolate or Leflunamide
71
What is eosinophilic granulomatosis with polyangiitis (Churg-Strauss)?
Eosinophilic granulomatosis with polyangiitis is an aesinophilic inflammation affecting small and medium sized vessels, most commonly in the respiratory tract and skin
72
How will EGPA most commonly present?
- Nervous system dysfunction - Skin irritation - Late onset asthma - Granulomatous inflammation
73
How can EGPA be distinguished from GPA?
EGPA will have a high eosinophil count and is a C-ANCA predominant condition
74
What is the predominant antibody in EGPA?
C-ANCA
75
How is remission induced in organ threatening EGPA?
Steroids + Cyclophosphamide
76
How is remission maintained in organ threatening EGPA?
Azathioprine/Methotrexate/Mepolizumab/ Rituximab
77
How is remission induced in non-organ threatening EGPA?
Steroids
78
How is resistant, non-organ threatening EGPA managed?
Mepolizumab - IL5 inhibitor
79
What is Henoch-Schönlein purpura (IgA Vasculitis)?
This is an acute IgA-mediated generalised vasculitis involving the small vessels of the skin, the GI tract, the kidneys, the joints, and rarely, the lungs and rarely, the CNS
80
Who is most likely to get Henoch-Schönlein purpura?
HSP occurs mostly in children aged 2-11
81
What is thought to cause HSP?
It is thought to be caused by a preceding URT infection, pharyngeal infection or GI infection, with group A streptococcus being the most common causative infection, occurring 1-3 weeks before HSP develops
82
How does HSP usually present?
- Purpuric rash, typically on the buttocks and lower limbs - Colicky abdominal pain - Bloody diarrhoea - Joint pain ± Swelling - Renal involvement (50%)
83
How is HSP usually managed?
HSP is usually self limiting and symptoms tend to resolve within 8 weeks, however, relapses may occur for months to years
84
How is resistant HSP treated?
Steroids and immunosuppressive drugs urinalysis for renal involvement
85

Musculoskeletal (123 decks)

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