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MSK + Rheumatology > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis?

A

An inflammatory disorder of blood vessel walls.
- inflammation & necrosis of blood vessel walls with subsequent impaired blood flow.

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2
Q

Briefly describe the pathophysiology of vasculitis.

A

Inflammation and necrosis of blood vessel walls with subsequent impaired blood flow resulting in:

  1. Vessel wall destruction - aneurysm, rupture and stenosis:
    * Resulting in perforation and haemorrhage into tissues
  2. Endothelial injury:
    * Resulting in thrombosis + ischaemia/infarction of dependent tissues
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3
Q

According to which 2 factors is vasculitis classified?

A
  1. Size of the blood vessel involved.
  2. Presence or absence of anti-neutrophil cytoplasmic antibodies (ANCA).
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4
Q

What does ANCA stand for?

A

Anti-neutrophil cytoplasmic antibodies.

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5
Q

What are the 3 categories of vasculitis classified according to size?

A
  1. Large-vessel vasculitis:
    - Refers to the aorta and its major tributaries
  2. Medium-vessel vasculitis:
    - Refers to medium and small-sized arteries and arterioles
  3. Small-vessel vasculitis:
    - Refers to small arteries, arterioles, VENULES and capillaries
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6
Q

Give 2 examples of medium-vessel vasculitis.

A
  1. Classical polyarteritis nodosa (PAN)
  2. Kawasaki’s disease
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7
Q

Give 2 examples of ANCA-associated small-vessel vasculitis.

A
  1. Microscopic polyangitis
  2. Granulomatosis with polyangitis
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8
Q

Give 2 examples of ANCA-negative small-vessel vasculitis.

A
  1. Essential cryoglobulinaemia
  2. Cutaneous leucocytoclastic vasculitis
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9
Q

Which 2 features are found in ALL forms of vasculitis?

A
  1. Raised ESR
  2. Anaemia
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10
Q

Are the types of vasculitis common?

A

ALL RARE except giant cell (temporal) arteritis.

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11
Q

Give an infective condition associated with vasculitis (risk factor).

A

Subacute infective endocarditis

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12
Q

Giv 3 non-infective conditions associated with vasculitis (risk factors).

A

Non-infective:
1. Vasculitis with RA
2. SLE
3. Scleroderma
4. Polymyositis/dermatomyositis
5. Good pasture syndrome and Inflammatory bowel disease (UC/Crohn’s)

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13
Q

Give 2 examples of large-vessel vasculitis.

A
  1. Giant cell arteritis/polymyalgia rheumatic
  2. Takayasu’s arteritis
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14
Q

Describe the epidemiology of large-vessel vasculitis.

A
  1. Affects those > 50 YO.
  2. More common in FEMALES than males
  3. Systemic disease of the elderly - incidence increases with age
  4. Associated with polymyalgia & RA
  5. In general - it’s rare; GCA is the most common type
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15
Q

What is Polymyalgia Rheumatica (PMR)?

A

A condition that causes pain, stiffness and inflammation in the muscles around the shoulders, neck and hips.

Separate condition to GCA - but usually co-exist

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16
Q

Give 2 risk factors for PMR.

A
  1. SLE
  2. Polymyositis/dermatomyositis
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17
Q

Describe the clinical presentation of polymyalgia rheumatica (PMR).

A

Sudden onset of severe pain and stiffness of the shoulders
and neck, and of the hips and lumbar spine; a limb girdle
pattern.

Symptoms are worse in the morning, lasting from 30 mins -
several hours

Mild polyarthritis of peripheral joints.

1/3rd experience: fatigue, fever, weight loss, depression.

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18
Q

Investigations for Polymyalgia Rheumatica (PMR).

A
  1. Clinical history is usually diagnostic and the patient is ALWAYS OVER 50
  2. BOTH ESR & CRP RAISED - diagnostic
  3. ANCA negative
  4. Serum alkaline phosphatase raised
  5. Mild normochromic, normocytic anaemia may be present
  6. Temporal artery biopsy:
    * Shows giant cell arteritis in 10-30% cases
  • Note: creatinine kinase is normal - helps to distinguish from myositis / myopathies
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19
Q

Treatment for Polymyalgia Rheumatica (PMR).

A
  1. Corticosteroids with PPI + Alendronate + Ca2+ + Vit. D
    - e.g. Oral Prednisolone
    - Give a dramatic reduction of symptoms of PMR within
    24-48 hours of starting treatment
  • PPI e.g. lansoprazole = GI protection due to long-term use of steroids
  • Alendronate = bisphophonate for osteroporosis prevention dye to steroids
  • Ca2+ and Vit. D = bone protection
  • If improvement doesn’t occur: diagnosis should be questioned
  • Decrease dose slowly
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20
Q

Define giant cell arteritis (GCA).

A

Inflammatory granulomatous arteritis of large CEREBRAL ARTERIES as well as other large vessels e.g aorta, which occurs in association with PMR

*Arteritis = inflammation of the lining of the arteries

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21
Q

Pathophysiology of GCA:
In GCA, which layers of the artery are affected?
What is the net effect of this?

A

Tunica media + interna.

Arteries become inflamed, thickened, cause narrowing of the lumen and can obstruct blood flow.

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22
Q

Which arteries are commonly affected in GCA?

A
  1. Cerebral arteries
    - Common temporal (splits into frontal + temporal)
    - Linguinal / facial
  2. Opthalmic arteries
    - Cilliary (to retina)
23
Q

Give 5 cranial-related symptoms of GCA.

A
  1. Severe headaches
    - Abrupt temporal unilateral headaches
  2. Scalp tenderness
  3. Jaw claudication (when eating)
  4. Tenderness + swelling of one or more temporal or occipital arteries
    - Visible dilation of temporal artery on side of face
  5. SUDDEN PAINLESS VISION LOSS - EMERGENCY
    - Arteritic anterior ischaemic optic neuropathy - optic disc is very pale/swollen
24
Q

Give 3 non-cranial-related symptoms of GCA.

A
  1. Malaise, lethargy, chronic low grade fever
  2. Associated symptoms of PMR
  3. Dyspnoea, morning stiffness and unequal or weak pulses
25
Q

What might you find on clinical investigation in someone with giant cell arteritis?

A
  1. Palpable and tender temporal arteries with reduced pulsation.
  2. Sudden monocular visual loss, the optic disc is pale and swollen.
26
Q

What is the diagnostic criteria for GCA?

A

Diagnostic criteria - 3 or more of:

  1. Age > 50
  2. New headache
  3. Temporal artery tenderness or decreased pulsation
  4. ESR raised
  5. Abnormal artery biopsy - inflammatory infiltrates present
27
Q

What investigations might you do in someone who you suspect has giant cell arteritis?

A
  1. Bloods:
    - Normochromic, normocytic anaemia
    - HIGH inflammatory markers - ESR and CRP
    - ANCA negative
    - Serum alkaline phosphatase may be raised
  2. Temporal artery biopsy - DIAGNOSTIC!!
    - Should be taken BEFORE or within 7 days of starting high dose
    corticosteroids
    * Lesions are patchy so take a big chunk!
  3. Ultrasound
    - Halo sign on US of temporal and axillary artery
28
Q

Investigations for GCA: Temporal Artery Biopsy - what would you observe?

A

Lesions are patchy so take a big chunk!

  • Histological features:
    1. Cellular infiltrates of CD4+ T lymphocytes, macrophages and giant cells (may not be visible in all cases) in the vessel wall
    = tightly-packed as if 1 giant cell
  1. Granulomatous inflammation of the intima & media
  2. Breaking up of the internal elastic lamina
29
Q

What cells might you see on a histological slide taken from someone with GCA?

A

Neutrophils and giant cells.

30
Q

Describe the treatment for GCA.

A
  1. High-dose Corticosteroids RAPIDLY with PPI + Alendronate + Ca2+ + Vit. D

e.g. PREDNISOLONE to stop vision loss
- Gradual reduction of steroids over 12-18 months

  • PPI e.g. lansoprazole = GI protection due to long-term use of steroids
  • Alendronate = bisphophonate for osteroporosis prevention/prophylaxis due to steroids
  • Ca2+ and Vit. D = bone protection
  1. Immunosuppressants that are used include:
    - Cyclophosphamide
    - Methotrexate
    - Azathioprine
    - Rituximab and other monoclonal antibodies
  2. Monitor treatment progress by looking at ESR/CRP (should fall).
31
Q

Define Polyarteritis nodosa (PAN).

A

A rare multi-system disorder characterised by widespread inflammation, weakening, and damage to small and medium-sized arteries.

32
Q

Pathophysiology of Polyarteritis nodosa: what is the underlying pathology of PAN?

A

A vasculitis - fibrinoid necrosis of vessel walls with microaneurysm formation, thrombosis and infarction in medium sized arteries.

33
Q

Give 3 risk factors for PAN.

A
  1. Male
  2. Hepatitis B
  3. RA, SLE, scleroderma
34
Q

Which vasculitis is most associated with Hep. B?

A

Polyarteritis nodosa (PAN).

35
Q

List some general + systemic features of polyarteritis nodosa (PAN).

A

General - fever, malaise, weight loss, myalgia.

Skin - skin lesions, purpura, livedo reticularis (a mottled, purplish, lace like rash), ulcers, gangrene.

Cardiac - angina, HF, pericarditis.

Renal - hypertension, haematuria, renal failure.

GI - pain or perforation, malabsorption.

GU - orchitis.

Neuro - Mononeuritis multiple (Numbness, tingling, abnormal / lack of sensation and inability to move part of the body)

36
Q

What investigations would you carry out in polyarteritis nodosa (PAN)?

A
  1. Bloods:
    - Anaemia
    - WCC raised
    - ESR + CRP raised
    - ANCA negative (it’s very rarely positive)
  2. Angiography:
    * Demonstrates micro-aneurysms in hepatic, intestinal or renal vessels
    - “String of beads” pattern along the artery
  3. Biopsy, of kidney in particular
    - To look for hypertension and other damage - can be diagnostic
37
Q

How would you treat polyarteritis nodosa (PAN)?

A
  1. BP control
    - ACEi e.g. Ramipril
  2. Corticosteroids with Immunosuppressants
    - Prednisolone with Azethioprine / Cyclophosphamide
  3. Hep. B should be treated with an antiviral after initial treatment with steroids
38
Q

what size vessels does ANCA vasculitis affect?

A

small

39
Q

what are the two ANCA associated vasculitides?

A

p-ANCA: microscopic polyangiitis, eosinophillic, UC, primary schlerosing cholangitis
c-ANCA: granulomatosis with polyangiitis (aka wegeners)

40
Q

eosinophilic granulomatosis presents similarly to

A

allergy

asthma

41
Q

What is Henoch-Schoenlein purpura?

A

IgA vasculitis (small-vessel vasculitis)

  1. Rash
  2. Abdominal pain
  3. Glomerulonephritis
  4. Arthritis/arthralgia
42
Q

Microscopic polyangiitis affects only the?

A

Kidney and lung

43
Q

What is Granulomatosis with polyangitis (Wegener’s granulomatosis)?

A

Granulomatosis with polyangiitis is a small vessel vasculitis.

It was previously known as Wegener’s granulomatosis.

44
Q

Is granulomatosis with polyangitis associated with c-ANCA or p-ANCA?

A

c-ANCA.

45
Q

What organ systems can be affected by granulomatosis with polyangitis?

A
  1. URT.
  2. Lungs.
  3. Kidneys.
  4. Skin.
  5. Eyes.
46
Q

What is the affect of granulomatosis with polyangitis on the URT?

A
  • Sinusitis.
  • Otitis.
  • Nasal crusting.
47
Q

What is the affect of granulomatosis with polyangitis on the lungs?

A
  • Pulmonary haemorrhage/nodules.
  • Inflammatory infiltrates are seen on X-ray.
48
Q

What is the affect of granulomatosis with polyangitis on the kidney?

A

Glomerulonephritis.

49
Q

What is the affect of granulomatosis with polyangitis on the skin?

A

Ulcers.

50
Q

What is the affect of granulomatosis with polyangitis on the eyes?

A
  • Uveitis.
  • Scleritis.
  • Episcleritis.
51
Q

Describe the clinical presentation of granulomatosis with polyangitis.

A

Classic sign on exams: saddle shaped nose
Epistaxis
Crusty nasal/ ear secretions 🡪 hearing loss
Sinusitis
Cough, wheeze, haemoptysis

52
Q

Diagnosis of granulomatosis with polyangitis.

A

High eosinophils on FBC.
Histology shows granulomas.
Presence of c-ANCA.

53
Q

What is the treatment for granulomatosis with polyangitis?

A
  • If severe: high dose steroids with immunosuppressants e.g. cyclophosphamide
  • If non-end organ threatening: moderate steroids.

MSK + Rheumatology (22 decks)

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