Vasculitis Flashcards

1
Q

What is vasculitis?

A

Inflammation of blood vessels, often with ischaemia, necrosis and organ inflammation (can affect any vessel)

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2
Q

What do the manifestations of vasculitis depend on?

A

Size and location of involved vessel, and the degree of organ dysfunction and inflammation

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3
Q

What is the prognosis of untreated small vessel vasculitis after two years?

A

90%

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4
Q

What causes primary vasculitis?

A

Results from inflammatory response that targets vessel walls and has no known cause, sometimes auto-immune

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5
Q

What causes secondary vasculitis?

A

May be triggered by infection, drug or toxin, or may occur as part of another inflammatory disorder or cancer

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6
Q

What is the pathogenesis of vasculitis?

A

Activated dendritic cells express receptors and release inflammatory cytokines that promote T cell activation and vascular inflammation, causing granuloma formation and macrophage activation

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7
Q

What are the common symptoms for all types of vasculitis?

A

Fever, malaise, weight loss and fatigue

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8
Q

What are the two main causes of large vessel vasculitis?

A

Takayasu arteritis (TA) and giant cell arteritis (GCA)

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9
Q

What is the epidemiology of Takayasu arteritis?

A

People < 40 years old, commoner in female, more prevalent in Asian populations

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10
Q

What is the epidemiology of giant cell arteritis?

A

People > 50 years old, typically cause temporal arteritis but aorta and other large vessels may be affected, associated with polymyalgia rheumatica

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11
Q

What is the presentation of Takayasu arteritis?

A

Claudication
Bruits = most commonly in the carotid arteries
Blood pressure difference at extremities = pulseless disease

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12
Q

What are the presentations of giant cell arteritis?

A

Unilateral headache, scalp tenderness and jaw claudication
Temporal arteries prominent with reduced pulsation
Risk of blindness due to ischaemia of optic nerve

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13
Q

What investigations should be done for large vessel vasculitis?

A

ESR, PV and CRP (raised)
Temporal artery biopsy = skip lesions occur so biopsy may be negative
MR/CT angiogram, USS and PET CT

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14
Q

How should large vessel vasculitis be treated?

A

40-60mg prednisolone, steroid sparing agent may be used (e.g methotrexate), tocilizumab

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15
Q

What are the two main middle vessel vasculitis?

A

Polyarteritis nodosa = typically affects muscular arteries, causing aneurysms
Kawasaki’s disease = affects children < 5, febrile exanthematous disease typically affecting the coronary arteries

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16
Q

What are the two types of small vessel vasculitis?

A

ANCA associated vasculitis (AAV) and ANCA negative

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17
Q

What are some examples of AAVs?

A

Granulomatosis with polyangiitis (GPA), Eosinophilic granulomatosis with polyangiitis (EGPA), Microscopic polyangiitis (MPA)

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18
Q

What occurs in GPA?

A

Granulomatous inflammation of the respiratory tract, necrotising glomerulonephritis common

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19
Q

What occurs in EGPA?

A

Eosinophilic granulomatous inflammation of the respiratory tract, associated with asthma

20
Q

What occurs in MPA?

A

Necrotising vasculitis with few immune deposits, necrotising glomerulonephritis very common

21
Q

What is the epidemiology of GPA?

A

More common in northern Europeans and men, typically patients aged 35-55, constitutional symptoms and arthralgia common, diagnosed if 2/4 of American College of Rheumatology 1990 criteria are present

22
Q

What are some ENT features of GPA?

A

Sinusitis, nasal crusting, epistaxis, mouth ulcers, otitis media and deafness, sensorineural deafness, saddle nose (due to cartilage ischaemia)

23
Q

What are some respiratory symptoms of GPA?

A

Pulmonary infiltrates, cough, haemoptysis, diffuse alveolar haemorrhage, cavitating nodules on CXR

24
Q

What are some cutaneous and renal manifestations of GPA?

A
Cutaneous = palpable purpura, cutaneous ulcers
Renal = necrotising glomerulonephritis
25
Q

What are some feature of GPA that occur in the nervous system?

A

Mononeuritis multiplex, sensorimotor polyneuropathy, cranial nerve palsies

26
Q

What are some ocular features of GPA?

A

Conjunctivitis, episcleritis, uveitis, optic nerve vasculitis, renal artery occlusion, proptosis

27
Q

How does EGPA present?

A

Similar features to GPA = main differences are late onset asthma and a high eosinophil count

28
Q

How does MPA present?

A

Shares many features with GPA but respiratory features are rare

29
Q

What is an example of an ANCA negative small vessel vasculitis?

A

Henoch-Schonlein purpura (HSP) = acute IgA mediated disorder

30
Q

What occurs in HSP?

A

Generalised vasculitis of small vessels of skin, GI tract, kidneys, joints and rarely lungs and CNS

31
Q

Who tends to get HSP?

A

Over 75% of cases are in children aged 2-11

32
Q

What normally precedes HSP?

A

UTI, pharyngeal infection or GI infection = most commonly group A Strep, preceding illness occurs 1-3 weeks earlier

33
Q

How does HSP present?

A

Purpuric rash typically over buttocks and limbs, colicky abdominal pain, bloody diarrhoea, joint pain +/- swelling, renal involvement

34
Q

What is the prognosis of HSP?

A

Usually self limiting = resolves within 8 weeks
Relapses may occur for months to years
Must perform urinalysis screen for renal involvement

35
Q

What investigations can be done for small vessels vasculitis?

A

Urine dipstick
FBC, liver and renal profile, inflammatory markers
ANCA and specific antibodies, connective tissue screening, compliment levels
CT scan
Nerve conduction tests and tissue biopsy

36
Q

What is the prognosis of untreated AAVs?

A

Usually fatal

37
Q

What is the treatment for localised/early systemic AAVs?

A

Methotrexate + steroids

38
Q

What is the treatment for generalised/systemic AAVs?

A

Cyclophosphamide + steroids (1st line)
Ritiximab + steroids (alternative)
Plasma exchange if creatine > 500
Followed with azathioprine (or alternatives e.g methotrexate)

39
Q

What is the treatment for refractory AAVs?

A

IV immunoglobulin, ritiximab

40
Q

What are ANCAs?

A

Anti-Neutrophil Cytoplasmic Antibodies = auto-antibodies against antigens in the cytoplasm of neutrophil granulocytes

41
Q

What is used to detect ANCA and differentiate patterns?

A

Immunofluorescence

42
Q

What are the different ANCA patterns present in AAVs?

A
GPA = cANCA
EGPA = pANCA
MPA = pANCA
43
Q

Do ANCA, anti-PR3 and anti-MPO stay constant in disease?

A

No = vary with disease activity

44
Q

What are AAVs associated with?

A

Formation of immune complexes

45
Q

What may happen to c3/4 levels during active disease?

A

Levels may fall as complement is consumed