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Year 2 Clinical > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

Define vasculitis?

A

Inflammation of blood vessels, often with ischaemia and necrosis, thrombosis and organ inflammation

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2
Q

Areas affected by vasculitis?

A

Any blood vessel, inc. capillaries

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3
Q

Define primary and secondary vasculitis?

A

Primary: results from an inflammatory response that targets the vessel walls and has an UNKNOWN CAUSE; it is sometimes autoimmune

Secondary: may be triggered by an infection, drug, toxin or it may occur as part of another inflammatory disorder of cancer, e.g: paraneoplastic vasculitis assoc. with cancer

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4
Q

Pathophysiology of vasculitis?

A
  1. Activated dendritic cells release cytokines that activate T cells and cause vascular inflammation
  2. T cells promote inflammation, GRANULOMA formation and macrophage activation
  3. Macrophages release mediators that cause progressive vascular inflammation, endothelial damage and intimal hyperplasia
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5
Q

Main classifications of vaculitis?

A

Large-vessel, inc. Takayasu arteritis and giant cell arteritis

Medium-vessel, inc. polyarteritis nodosa and Kawasaki disease

Small-vessel is split into two categories

PICTURE 1

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6
Q

2 categories of small vessel vasculitis?

A

ANCA-associated small-vessel vasculitis (AAV):
• Granulomatosis with polyangiitis (GPA)
• Eosinophilic granulomatosis with polyangiitis (EGPA)
• Microscopic polyangiitis (MPA)

Immune complex small-vessel vasculitis:
• Henoch-Schönlein (common)
• Others

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7
Q

Systemic symptoms common to all vasculitides?

A

Fever
Malaise
Fatigue
Weight loss

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8
Q

Two main causes of large vessel vasculitis and histopathology?

A

Takayasu arteritis (TA)

Giant Cell arteritis (GCA)

There is GRANULOMATOUS infiltration of the large vessel walls

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9
Q

Occurrence of takayasu arteritis?

A

Tends to affect those <40 YEARS

More common in FEMALES

It is much more prevalent in Asian populations

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10
Q

Occurrence of GCA?

A

Generally affects those > 50 YEARS

Typically causes temporal arteritis but the aorta and other large vessels may be affected

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11
Q

Signs on examination of large vessel vasculitis?

A
  • Bruit (usually if carotid artery is affected)
  • BP difference of extremities
  • Claudication (in Takayasu, this occurs suspiciously in younger people)
  • Carotydinia or vessel tenderness (painful to touch)
  • Hypertension
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12
Q

Occurrence of temporal arteritis?

A

Assoc. with polymyalgia rheumatica

About 50% with GCA have PMR, and about 15% of individuals with PMR develop GCA

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13
Q

Classical symptoms of temporal arteritis?

A
  • Unilateral temporal headache
  • Scalp tenderness
  • Jaw claudication
  • Prominent temporal arteries with reduced pulsation
  • Pain over temporal artery
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14
Q

Risks with temporal arteritis?

A

Of blindness due to ischaemia of the optic nerve

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15
Q

Ix of temporal arteritis?

A

Inflammatory markers (ESR, PV and CRP) are raised

Temporal artery biopsy shows granulomas; biopsy may be -ve as there are SKIP LESIONS

MR angiogram can show stenosis/thickening/aneurysm

PET CT can shows increased metabolic activity of inflammatory cells

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16
Q

Management of temporal arteritis?

A

If there are no visual problems, 40 mg prednisolone gradually reduced over 18 months to 2 years

If there are visual problems, 60 mg prednisolone gradually reduced over 18 months to 2 years

Occasionally, if the patient struggles with a reduced steroid dose, methotrexate/azathioprine may be considered

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17
Q

Types of medium vessel vasculitis?

A

Kawasaki disease

Polyarteritis nodosa

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18
Q

What is Kawasaki disease?

A

Seen in CHILDREN, usually < 5 years, often following an INFECTION

Causes vasculitis of various vessels but can affect CORONARY ARTERIES, leading to aneurysm development

Tends to be self-limiting

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19
Q

What is polyarteritis nodosa?

A

Characterised by necrotising inflammatory lesions that affect arteries at vessel bifurcations, leading to microaneurysm and aneurym formation, and often affecting skin, gut and kidneys

Assoc. with HEP B

20
Q

What were the different types of small vessel vaculitis previously known as?

A

GPA was previously known as Wegener’s granulomatosis

EGPA was previously known as Churg-Strauss syndrome

Microscopic polyangiitis has not changed name

21
Q

Pathology of GPA?

A

Granulomatous inflammation of respiratory tract, small and medium vessels

Necrotising GLOMERULONEPHRITIS common

22
Q

Pathology of EGPA?

A

Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels; increased eosinophils in blood and tissues

Assoc. with asthma

23
Q

Pathology of MPA?

A

Necrotising vasculitis with few immune deposits

Necrotising GLOMERULONEPHRITIS very common

24
Q

Ecologic factors in development of AAV?

A

Bacteria - Staph. aureus carriage in the nose increases risk of GPA

Genetic factors

Environmental, e.g: silica

Drugs, e.g: hydralazine

25
Q

Occurrence of GPA?

A

More common in northern european descent

SLIGHTLY MORE COMMON in MALES

Can occur at any age, usually between 35-55 years

26
Q

GPA classification critera?

A

Must have at least 2 of the 4 criteria

27
Q

ENT features of GPA?

A

There are the cardinal features:
• Sinusitis (stuffiness and discharge)
• Nasal crusting
• Epistaxis (nosebleed)

• Mouth ulcers

  • Sensorineural deafness
  • Otitis media (infection of middle ear) and deafness

• “Saddle nose” due to cartilage ischaemia

28
Q

Respiratory signs of GPA?

A

Cough and haemoptysis
Diffuse alveolar hemorrhage (emergency)

Pulmonary infiltrates 
Cavitating nodules (usually, > 1) on CXR
29
Q

Cutaneous signs of GPA?

A

NON-BLANCHING, raised/palpable purpura

These can join together and ulcerate

30
Q

Renal issues in GPA?

A

NECROTISING GLOMERULONEPHRITIS manifests as blood and protein in the urine

31
Q

Nervous system manifestations of GPA?

A

Mononeuritis multiplex commonly presents with wrist/foot drop

Sensorimotor polyneuropathy

Cranial nerve palsies (cannot move eye either due to granuloma invasion of a muscle or ischaemia of the nerve)

32
Q

Ocular manifestations of GPA?

A
  • Conjunctivitis, episcleritis, uveitis
  • Optic nerve vasculitis
  • Retinal artery occlusion
  • Proptosis (eye is pushed outwards by a large granuloma)
33
Q

Main differences between GPA and EGPA?

A

Late onset asthma

High eosinophil count

It does not have the same ENT signs that GPA does, e.g: nasal cartilage, collapse is not common

34
Q

Criteria for EGPA?

A

Should have 4 or more of:
• Asthma (wheezing, expiratory rhonchi)

  • Eosinophilia
  • Paranasal sinusitis
  • Pulmonary infiltrates (may be transient)
  • Histological proof of vasculitis with extravascular eosinophils
  • Mononeuritis multiplex or polyneuropathy
35
Q

What is ANCA?

A

Anti-neutrophil cytoplasmic antibodies - group of antibodies in the cytoplasm of neutrophil grnaulocytes

36
Q

Types of ANCA?

A

cANCA (cytoplasmic) is very specific to GPA

pANCA (perinuclear) is less specific and are present in MPA and EGPA

37
Q

Other antibodies?

A

Anti-PR3 is very specific for GPA

Anti-MPO is seen in EGPA and MPA; it is not as useful for GPA

38
Q

Antibodies present in GPA?

A

cANCA and anti-PR3

39
Q

Antibodies present in EGPA?

A

pANCA and anti-MPO

40
Q

How can disease be monitored?

A

ANCA, anti-PR3 and anti-MPO levels all vary with disease activity

Complement is consumed during active disease so C3/4 may fall

41
Q

Management of AAV?

A

Localised/early systemic:
• Methotrexate + steroids

Generalised/systemic:
• 1st line - CYCLOPHOSPHAMIDE + STEROIDS
• Alternative - Rituximab + steroids
• This is followed by azathioprine; alternative inc. methotrexate, etc

Refractory (severe):
• IV Ig
• Rituximab

42
Q

What is Henoch-Schönlein purpura (HSP)?

A

AKA acute IgA mediated disorder; it is a type of immune complex small-vessel vasculitis that tends to occur in CHILDREN between 2-11 YEARS (RARE in INFANTS)

It is a generalised vasculitis inv. small vessels of the skin, GI tract, kidneys and joints; rarely, vessel of the lungs and CNS

43
Q

Triggers of HSP?

A

Most have a preceding URT, pharyngeal or GI infection. which usually occurs 1-3 weeks beforehand

Most common is group A Strep.

44
Q

Presentation of HSP?

A

Purpuric rash typically over buttocks and lower limbs, sparing the upper body

Colicky abdominal pain

Bloody diarrhoea

Joint pain +/- swelling

Renal involvement (50%)

45
Q

Management of HSP?

A

Usually, SELF-LIMITING and symptoms tend to resolve within 8 weeks; relapses may occur for months-years but will eventually stop

MUST PERFORM URINALYSIS to screen for renal inv.

Year 2 Clinical (119 decks)

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