Disease Profiles: Vasculitis Flashcards
Define early systemic ANCA-associated small vessel vasculitis
Any systemic involvement, no organs affected and not life-threatening
Describe the prognosis of Henoch-Schonlein Purpura (HSP)
Usually self-limiting within 8 weeks, may be relapses
Which form of imaging would you use to investigate Takayasu arteritis?
MR angiogram
What patient group is most likely to develop Henoch-Schonlein Purpura (HSP)?
Children 2-11, following a preceding infection (1-3 weeks)
Why must urinalysis be performed in Henoch-Schonlein Purpura (HSP)?
To check for renal involvement
Which form of vasculitis should always be considered in the differential diagnosis of a new-onset headache in patients 50+ with elevated ESR, CRP or PV?
Giant cell arteritis
Why does a positive temporal artery biopsy for giant cell arteritis have 100% specificity but relatively low sensitivity?
Due to the presence of skip lesions - biopsy may be taken from a normal segment
What blood values would you expect to be increased in ANCA-associated small vessel vasculitis?
ESR, PV and CRP (+ deranged U+E if renal involvement)
What causes ‘saddle nose’ in granulomatosis with polyangiitis?
Cartilage damage from ischaemia
Describe the systemic symptoms common to all vasculitides
Fever, malaise, weight loss, fatigue
How would you manage a patient with Takayasu arteritis?
Start with prednisolone 40-60mg daily, use steriod sparing agents if needed e.g. leflunamide, methotrexate
Which renal complication is common in granulomatosis with polyangiitis and microscopic polyangiitis?
Necrotising glomerulonephritis
What blood values would you expect to be raised in polymyalgia rheumatica?
ESR, plasma viscosity, CRP
What would a temporal biopsy of a patient with giant cell arteritis show?
Transmural arterial inflammation, patchy infiltration by lymphocytes, macrophages and multinucleated giant cells, vessel wall thickening which may cause distal ischaemia
Which patient group is most likely to develop giant cell arteritis?
Patients in late 60s+
What is granulomatosis with polyangiitis?
Granulomatous inflammation affecting small and medium sized vessels in the upper and lower respiratory tract, eyes and/or kidneys (necrotising glomerulonephritis common)
Which ANCA-associated small vessel vasculitis is associated with MPO-ANCA/pANCA in ∼70% of cases?
Microscopic polyangiitis
Which patient group is most likely to develop polymyalgia rheumatica?
> 50 years, higher incidence in northern regions, GCA association
What are anti-neutrophil cytoplasmic antibodies (ANCAs)?
Auto-antibodies against antigens in the cytoplasm of neutrophil granulocytes
What causes bruit, reduced pulses and blood pressure difference of extremities in Takayasu arteritis?
Vascular stenosis and aneurysms
Describe the ocular manifestations of granulomatosis with polyangiitis
Conjunctivitis, episcleritis, uveitis, optic nerve vasculitis, retinal artery occlusion, proptosis
What is the first line imaging investigation for giant cell arteritis?
Temporal artery USS
What is the treatment for refractory ANCA-associated small vessel vasculitis?
IV immunoglobins
Rituximab
Which vasculotide is polymyalgia rheumatica often associated with?
Giant cell arteritis
Name the two categories of large vessel vasculitis
Temporal (giant cell) arteritis and Takayasu arteritis
What is the first line treatment and subsequent maintenance therapy for systemic ANCA-associated small vessel vasculitis?
Cyclophosphamide and steroids, followed by azathioprine for maintenance
Plasma exchange if creatinine > 500
What are the main differences between eosinophilic granulomatosis with polyangitis and microscopic polyangiitis?
Late onset asthma, high eosinophil count and ANCA specificity
Which blood values would you expect to be elevated in Takayasu arteritis or GCA?
ESR, PV and CRP
Name the ANCA-associated small vessel vasculitides
Granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangitis, microscopic polyangiitis
What is microscopic polyangiitis?
Necrotising vasculitis of small vessels with few immune deposits, typically with pulmonary, renal and skin involvement
Describe the articular manifestations of polymyalgia rheumatica
Proximal myalgia of the hip and shoulder girdles with accompanying morning stiffness that lasts for more than 1 hour
Stiffness improves with movement
Usually symmetrical
Movement reduced but muscle strength normal
Which ANCA-associated small vessel vasculitis is associated with PR3-ANCA/c-ANCA in ∼90% of cases
Granulomatosis with polyangiitis
Define vasculitis
Inflammation of the blood vessels (arteries, arterioles, veins, venues, capillaries)
What is secondary vasculitis?
Vasculitis triggered by e.g. an infection, drug or toxin, may occur as part of another inflammatory disorder or cancer
Describe the ENT manifestations of granulomatosis with polyangiitis
Sinusitis, nasal crushing, epistaxis, mouth ulcers, sensorineural deafness, otitis media and deafness, ‘saddle nose’, subglottic inflammation
Define localised ANCA-associated small vessel vasculitis
Upper and/or lower respiratory tract disease without any other systemic involvement or constitutional symptoms
What is large vessel vasculitis?
Primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches
Which ANCA-associated small vessel vasculitis is associated with MPO-ANCA/pANCA in ∼40% of cases?
Eosinophilic granulomatosis with polyangitis
What is the first line treatment and subsequent maintenance therapy for generalised ANCA-associated small vessel vasculitis?
Cyclophosphamide and steroids, followed by azathioprine for maintenance
Define systemic ANCA-associated small vessel vasculitis
Renal (creatinine < 500) or other vital organ failure
What does anaemia indicate in ANCA-associated small vessel vasculitis?
Chronic disease
Which form of vasculitis presents with systemic symptoms, claudication of the upper and lower limbs, and can progress to bruit and reduced pulses?
Takayasu arteritis
Describe the management of polymyalgia rheumatica
Start at prednisolone 15mg daily, reduce dose over 18 months - 2 years
What would an MR angiogram show in Takayasu arteritis?
Thickened vessel walls and stenosis
What is Henoch-Schonlein Purpura (HSP)?
IgA mediated generalised vasculitis (not ANCA associated) involving small vessels of the skin, GI tract, kidneys, joints, rarely the lungs and CNS
What is the first line treatment for early systemic ANCA-associated small vessel vasculitis?
Methotrexate and steriods
Define refractory ANCA-associated small vessel vasculitis
Progressive disease unresponsive to steroids + cyclophosphamide
What is primary vasculitis?
Vasculitis that results from an inflammatory response that targets the vessel walls and has no known cause
Which form of vasculitis presents with a unilateral acute temporal headache with focal tenderness on direct palpation, jaw claudication, visual disturbances/loss and tender enlarged non-pulsatile temporal arteries? (+ systemic symptoms)
Giant cell arteritis
Describe the systemic manifestations of polymyalgia rheumatica
Fatigue, anorexia, weight loss, fever
Describe the cutaneous manifestations of granulomatosis with polyangiitis and eosinophilic granulomatosis with poluangitis
Palpable purpura, oral/cutanous ulcers
What is the first line treatment for localised ANCA-associated small vessel vasculitis?
Methotrexate + steroids
How would you manage a patient with giant cell arteritis?
Start with prednisolone 40-60mg daily, gradual reduction over 18 months-2 years
If early relapse - leflunamide, methotrexate
If resistant/relapsing - toclizumab
Define generalised ANCA-associated small vessel vasculitis
Renal (creatinine < 500) or other organ threatening
What does decreased complement (C3+C4) indicate in ANCA-associated small vessel vasculitis?
Active disease
Describe the respiratory manifestations of granulomatosis with polyangiitis
Cough, haemoptysis, pulmonary infiltrates, diffuse alveolar haemorrhage, cavitating nodules on CXR
What is the diagnostic test for ANCA-associated small vessel vasculitis?
Biopsy
Which organism is most commonly associated with the infections that usually precede Henoch-Schonlein Purpura (HSP)?
Group A Strep (GAS) - URT, pharyngeal, GI
What is the diagnostic investigation for giant cell arteritis?
Temporal artery biopsy
What is polymyalgia rheumatica?
Relatively common chronic inflammatory condition of unknown aetiology that affects elderly individuals
Which patient group is most likely to develop an ANCA-associated small vessel vasculitis?
Males (0.5x higher) age 35-55 years, Northern European
Which patient group is most likely to develop Takayasu arteritis?
Females < 40 years, more prevalent in Asian populations
Describe the clinical presentation of Henoch-Schonlein Purpura (HSP)
Purpuric rash typically over buttocks and lower limbs, colicky abdominal pain, bloody diarrhoea, joint pain +/- swelling, may be renal involvement
Which investigations would you perform in suspected giant cell arteritis where other results have been inconclusive and high clinical suspicion remains?
PET CT or CT angiogram
What causes jaw claudication in giant cell arteritis?
Ischaemia of the maxillary artery
