Vasculitis Flashcards
What is vasculitis?
inflammation of blood vessels
often with ischaemia/necrosis/organ inflammation
What type of blood vessel is affected by vasculitis?
can affect any blood vessel
=> arteries, arterioles, veins, venules, or capillaries
What is the difference between primary and secondary vasculitis?
Primary - autoimmune inflammatory response targets vessel walls and has no known cause
Secondary - triggered by an infection/drug/toxin OR another inflammatory disorder or cancer.
Describe the pathogenic changes that occur inside the blood vessel walls?
activated macrophages produce mediators progressive vascular inflammation endothelial damage disruption of internal elastic lamina intimal hyperplasia
What vessels are affected in small vessel vasculitis?
arterioles, capillaries and venules
What vessels are affected in medium vessel vasculitis?
arteries
Give examples of conditions which are classed as small vessel vasculitis
Cryoglobulinemic
IgA mediated
Hypocomplementemic urticarial
Give examples of medium vessel vasculitis
Polyarteritis nodosa
Kawasaki Disease
What type of vasculitis are Giant Cell and Takayasu arteritis?
Large vessel
What types of small vessel vasculitis are ANCA associated?
Microscopic polyangiitis
Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis
What symptoms are common to all types of vasculitis?
fever
malaise
weight loss
fatigue
What age group and gender are more likely to get Takayasu arteritis?
<40 years and commoner in females.
More prevalent in Asian populations.
What age group is most likely to get giant cell arteritis?
> 50 years
What features commonly present in large vessel vasculitis?
Bruit (usually carotid artery)
Blood pressure difference of extremities
Claudication
What other muscular condition is associated with temporal arteritis?
polymyalgia rheumatica
What symptoms are usually present in temporal arteritis?
Unilateral temporal headache
scalp tenderness
jaw claudication
Temporal arteries prominent with reduced pulsation.
What nerve is at risk of being damaged in temporal arteritis and what are the consequences?
Risk of blindness due to ischaemia of the optic nerve.
What investigations are used to diagnose temporal arteritis?
ESR, plasma viscosity and CRP raised
Temporal artery biopsy
(“skip lesions” occur so biopsy may be negative)
MR angiogram or PET CT
What treatment is used for temporal arteritis?
40-60mg prednisolone.
Steroid sparing agents may be considered.
What condition is characterised by granulomatous inflammation of the respiratory tract, small and medium vessels with common necrotising glomerulonephritis?
granulomatosis polyangiitis
What condition is characterised by granulomatous inflammation of respiratory tract and small/medium vessels and an association with asthma?
Eosinophilic granulomatosis with polyangiitis
Necrotising vasculitis with a few immune deposits, and necrotising glomerulonephritis is common in this condition.
Microscopic polyangiitis
What age group and what gender most commonly develop granulomatosis polyangiitis ?
Male-to-female ratio of 1.5 : 1
Typically age 35-55 years
What organ systems are involved in granulomatosis polyangiitis?
ENT Respiratory Skin Renal Nervous system Ocular
What are the criteria for a diagnosis of eosinophilic granunlomatosis with polyangiitis?
Asthma
Eosinophilia (>10% in peripheral blood)
Paranasal sinusitis
Pulmonary infiltrates (may be transient)
Histological proof of vasculitis with extravascular eosinophils
Mononeuritis multiplex or polyneuropathy
must have 4 or more
What are ANCA antibodies?
Anti-Neutrophil Cytoplasmic Antibodies (ANCAs)
auto-antibodies against antigens in the cytoplasm of neutrophil granulocytes
What test is used to detect ANCA?
Immunofluoresence
Can differentiate ANCA patterns
e.g. cANCA and pANCA
c-ANCA is associated with GPA and EGPA, whilst p-anca is associated with MPA. TRUE/FALSE?
FALSE
c-ANCA is associated with GPA
p-ANCA is associated with EGPA and MPA
Is PR3 or MPO linked to GPA?
PR3
MPO is high in EGPA and MPA
What happens to complement levels when disease is active?
Complement is consumed during active disease so C3/4 may fall
How is ANCA Associated Vasculitis managed?
Local/early systemic = Methotrexate + steroids
Generalised/systemic = Cyclophosphamide + steroids
Refractory = IV immunoglobulins / Ritixumab
What microbe usually causes the infection which precedes HSP?
group A streptococcus
What are the common symptoms of HSP?
Purpuric rash typically over buttocks and lower limbs
Colicky abdominal pain
Bloody diarrhoea
Joint pain +/- swelling
Renal involvement (50%)
Is HSP self limiting?
Usually self-limiting
Symptoms tend to resolve within 8 weeks
