Vasculitis Flashcards by Kieran Ruthven

Types of large vessel vasculitis (2)

Takayasu

- Giant Cell Arteritis

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Examples of medium vessel vasculitis (3)

1) Kawasaki
2) PAN
3) Isolated CNS vasculitis

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Examples of small vessel vasculitis (3)

1) Granulomatosis with polyangiitis (GPA -> Wegners)
2) Eosinophilic granulomatosis with polyangiitis (EGPA -> Churg-Strauss)
3) Microscopic polyangiitis

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Most common vasculitis in UK is…

GPA

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Skin features of ANCA+ vasculitis (4)

Infarcts, purpura, ulcer, gangrene

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Classical ENT symptom for GPA

Subglottic stenosis

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Treatment outline for localised vasculitis (2)

Steroids

- Immunosuppresants (methotrexate) if needed

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Generalised vasculitis treatment (2)

“Stronger” immunosuppresants such as cyclophosphamide + steroids

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Refractory vasculitis treatment options (2)

IV immunoglobulin

- Rituximab (targets CD20 on B-cells)

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Untreated small-vessel vasculitis has what % mortality rate after 2 years?

90%

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Primary vasculitis always has a known cause. True/false?

False - often no cause implicated. Compared to SECONDARY vasculitis which is always triggered by a factor (e.g. infection, drug, toxin)

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Asthma & eosinophilia vasculitis is most likely….

EGPA

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Granuloma without asthma or eosinophilia vasculitis most likely…

GPA

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Which large vessel vasculitis is common in Asian women <40?

Takayasu Arteritis

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Giant cell arteritis is a type of large/medium vessel vasculitis. Who does it usually affect?

Large vessel.

>50 year olds

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GCA can cause temporal arteritis, and also inflammation in which other major vessel?

Aorta

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What’s the common presenting feature to both TA and GCA? (3)

Bruits (most commonly in the carotids 80%), blood pressure difference in extremeties, carotodynia.

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Temporal arteritis is associated with bilateral/unilateral headache and which other classical symptom?

Unilateral headache - classical symptom is jaw claudication

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GCA is commonly associated with which other rheumatic condition?

PMR (50% of GCA will have PMR and 15% of PMR will develop GCA)

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Name a serious complication of GCA?

Blindness (due to optic nerve ischaemia)

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Management of GCA is with what dosage of what kind of steroid?

40-60mg of prednisolone

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GCA is usually associated with an occipital, sharp headache. True/false?

False (unilateral)

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GCA only causes inflammation of temporary arteries. True/false?

False (aorta as well)

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GCA is commoner in those <50 years old. True/false?

False (TA more common in younger <40 year olds, GCA >50 year olds)

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Treatment of GCA with steroids is likely to be lifelong. true/false?

False - only to settle inflammation

Steroids should only be started once a positive biopsy result has been obtained in GCA. True/false?

False

Methotrexate should be started at the same time as steroids in GCA. True/false?

False

Medium vessel vasculitis is most commonly seen in adults/children?

Children

Medium-vessel vasculitis commonly affects which arteries?

Coronary arteries

Polyarteritis nodosa is a form of which type of vasculitis?

Medium

Polyarteritis nodsoa typically affects vessels at which points?

Bifurcations

Polyarteritis nodosa is associated with which viral infection?

Hepatitis B

GPA is more common in women or men?

Men (especially northern Europeans)

GPA typically onsets between which age ranges?

35-55 years old

What are some ENT symptoms of GPA small-vessel vasculitis (4)

1) Sinusitis 2) Saddle nose (due to cartilage ischaemia) 3) Mouth ulcers 4) Hearing loss

What are some respiratory symptoms of GPA small-vessel vasculitis? (3)

1) Pulmonary infiltrates 2) Haemoptysis 3) Cavitating nodules

What are some renal symptoms of GPA small-vessel vasculitis (2)

1) Necrotising glomerulonephritis | 2) Protein + blood in urine

What are some CNS symptoms of GPA small-vessel vasculitis? (2)

1) Mononeuritis multiplex (also in EGPA) | 2) CN palsy

What are some ocular symptoms of GPA small-vessel vasculitis? (3)

1) Conjunctivis 2) Uveitis 3) Proptosis

What's the main difference between GPA and EGPA? (2)

In EGPA: 1) late onset asthma 2) high eosinophil count

Is butterfly rash a common feature of GPA?

No, it's a feature of SLE

Is hearing loss a feature of GPA?

Yes

``` Which of the following is NOT a feature of EGPA? A. nasal cartilage collapse B. late onset asthma C. eosinophilia D. mononeuritis multiplex E. purpuric rash ```

A. nasal cartilage collapse = this is GPA

ANCA antibodies are directed against what?

Neutrophil granulocytes

cANCA is positive/negative in GPA?

Positive

pANCA is positive in which type of vasculitis?

EGPA

Which ANCA type is positive in MPA?

pANCA

PR3 positive antibody is suggestive of which type of vasculitis?

GPA

MPO positive antibody is suggestive of which vasculitis?

MPA (also mildly EGPA)

Do ANCA/anti-PR3 and MPO vary with disease activity?

Yes

Is complement consumed during active vasculitis process?

Yes - C3 and C4 may fall

ANCA is positive in all vasculitis. True/false?

False (only some small vessel vasculitis)

ANCA is positive in all patients with GPA. True/false?

False - only cANCA

cANCA and anti-PR3 are associated with EGPA. True/false?

False - GPA

cANCA and anti-MPO are associated with GPA. True/false?

False - MPO is MPA

cANCA and anti-PR3 are associated with GPA. True/false?

True

Localised or early systemic vasculitis can be treated how (2)?

1) Methotrexate | 2) Steroids

Generalised or systemic vasculitis is treated how? (2)

1) Steroids 2) Cyclophosphamide OR 1) Rituximab 2) Steroids Both options with plasma clearing if creatinine >500

Refractory vasculitis is treated how? (2)

IVIG, rituximab

True or false: ANCA-associated vasculitis (AAV) is commoner in women?

False (commoner in men)

Nasal carriage of S. aureus may be a risk factor for AAV, true or false?

True

EGPA is the new term for Wegner's, true/false?

False - EGPA is new Churg-Strauss

Recurrent sinusitis is a common feature of EGPA. True/false?

False (EGPA is asthma and eosinophilia)

Renal disease is rare in GPA. True or false?

False (necrotising glomerulonephritis)

Is Henloch-Schlen purpura an ANCA positive vasculitis?

No, ANCA-negative

HSP is mediated by which immunoglobulin?

IgA

HSP is mostly preceded by which infection type?

URTI (commonly group-A streptococcous)

In HSP, which one test is absolutely essential and why?

Urinalysis for renal involvement