Vasculitis Flashcards
Types of large vessel vasculitis (2)
- Takayasu
- Giant Cell Arteritis
Examples of medium vessel vasculitis (3)
1) Kawasaki
2) PAN
3) Isolated CNS vasculitis
Examples of small vessel vasculitis (3)
1) Granulomatosis with polyangiitis (GPA -> Wegners)
2) Eosinophilic granulomatosis with polyangiitis (EGPA -> Churg-Strauss)
3) Microscopic polyangiitis
Most common vasculitis in UK is…
GPA
Skin features of ANCA+ vasculitis (4)
Infarcts, purpura, ulcer, gangrene
Classical ENT symptom for GPA
Subglottic stenosis
Treatment outline for localised vasculitis (2)
- Steroids
- Immunosuppresants (methotrexate) if needed
Generalised vasculitis treatment (2)
“Stronger” immunosuppresants such as cyclophosphamide + steroids
Refractory vasculitis treatment options (2)
- IV immunoglobulin
- Rituximab (targets CD20 on B-cells)
Untreated small-vessel vasculitis has what % mortality rate after 2 years?
90%
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)
Asthma & eosinophilia vasculitis is most likely….
EGPA
Granuloma without asthma or eosinophilia vasculitis most likely…
GPA
Which large vessel vasculitis is common in Asian women <40?
Takayasu Arteritis
Giant cell arteritis is a type of large/medium vessel vasculitis. Who does it usually affect?
Large vessel.
>50 year olds
GCA can cause temporal arteritis, and also inflammation in which other major vessel?
Aorta
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.
Temporal arteritis is associated with bilateral/unilateral headache and which other classical symptom?
Unilateral headache - classical symptom is jaw claudication
GCA is commonly associated with which other rheumatic condition?
PMR (50% of GCA will have PMR and 15% of PMR will develop GCA)
Name a serious complication of GCA?
Blindness (due to optic nerve ischaemia)
Management of GCA is with what dosage of what kind of steroid?
40-60mg of prednisolone
GCA is usually associated with an occipital, sharp headache. True/false?
False (unilateral)
GCA only causes inflammation of temporary arteries. True/false?
False (aorta as well)
GCA is commoner in those <50 years old. True/false?
False (TA more common in younger <40 year olds, GCA >50 year olds)
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