Vasculitis Flashcards
Name the three ANCA-associated small vessel vasculitis
Microscopic polyangiitis
Granulomatosis with polyangitis
Eosinophillic granulomatosis with polyangitis
what is the most common large vessel vasculitis
Giant cell arteritis
which vessels does giant cell arteritis affect
aorta and its major branches
how can giant cell arteritis present?
headache - new, temporal with tenderness, subacute onset, constant, no relief with analgaesics.
Scalp tenderness - hairbrushing is sore.
Visual - sudden unilateral blindess
Jaw claudication - may cause weight loss
symptoms of polymyalgia rheumatica
Constitutional upset
what are the complciations of giant cell arteritis
visual loss - can be irreversible. Acute ischaemic optic neuropathy. Sudden painless loss of vision often proceeded by amaurosis fugax
Vascular stenosis and aneurysms due to the vasculitis.
CVA - obstruction of internal carotid artery or vertebral arteries.
what would you find on clinical exam in a patient with GCA?
temporal artery asymmetry, thickening, loss of pulsatility and tenderness
what would a temporal artery biopsy show in GCA?
if positive would have interruption of the internal elastic lamina with infiltration of mononuclear cells into the vessel wall.
Caution - get skip lesions so a biopsy may not show signs of inflammation and diagnosis would be missed
Done after patients have commenced treatment.
what is the classical sign observed in a temporal artery USS in those with GCA
halo sign which is a hypoechogenic mural thickening that is observed in inflamed arteries
how do you treat GCA
high dose steroids - 1mg/kg/day - oral prednisilone. Maintained for a month at this high level then tapered down. TO be used for 12-18 months.
Would use IV if patient had visual symptoms.
Aspirin for ischaemic complications
what is Henoch-Schönlein Purpura (HSP)
who is it more common in
a small vessel vasculitis
Children but can be observed in adults
describe how HSP may present?
purpuric rash on the buttock, thighs and sometimes lower legs. Urticarial rash, petechiae, ulcers. Artralgia/arthtiris in the lower limb in a majority of cases.
can be triggered by a strep throat
name some complications of HSP
GI - pain, bleeding, diarrhoea
Renal - IgA nephropathy (more common in adults)
Urological - orchitis (inflammation of the testicles)
how do you manage HSP
exclude other causes of a cutaneous vasculitis: history of other diseases, immunology tests: ANCA, RF, ANA, PR3/MPO etc., virology.
Urinalysis/urine PCR to assess the extent of the disease.
Often don’t treat but can give corticosteroids for certain complications e.g. testicular torsion, GI disease.
Frequently self-limiting but does relapse in about 5-10% of patients.
what is the characteristic lesion found in granulomatosis with polyangiitis
granulomatous necrotising inflammatory lesions of the upper and lower respiratory tract and often a pauci-immune glomerulonephritis
what is the triad of disease that exists in GPA
Upper airway/ENT • Rhinitis • Chronic sinusitis • Chronic otitis media • Saddle nose deformity • Nasal septal perforation Lower Respiratory • Parenchymal nodules +/- cavitation • Alveolar haemorrhage Renal • Pauci-immune glomerulonephritis (rapidly-progressing)
Others - consitutional • Fatigue • Weight loss • Fever/sweats • Myalgia/arthralgia • Failure to thrive in the elderly
what are the two ways of testing ANCA
what is it used for?
ANCA are autoantibodies which are directed against the cytoplasmic constituents of neutrophils and monocytes
o Indirect immunofluorescence – gives cANCA or pANCA staining pattern
o ELISA for PR3/ MPO
PR3 and MPO are the most commonly observed antigens
Useful to support a diagnosis, prognostic information, assessing response to treatment and monitoring for early signs of relapse.
May be negative in some patients
what would cANCA with PR3 be suggestive of?
GPA
what would pANCA with strong MPO be suggestive of
MPA, or EGPA
if a patient had moderate GPA which drugs would you give them to induce remission?
prednisolone in addition to methotrexate and mycophenolate
if a patient had severe GPA which drugs would you give them to induce remission?
prednisolone in addition to either cyclophosphamide or rituximab.
name two drugs used to maintain remission in vasculitis
azathioprine, methotrexate
At low doses
