Vasculitis Flashcards
What is vasculitis?
Inflammation of the blood vessels. Often with ischaemia, necrosis and organ involvement.
Which blood vessels does vasculitis affect?
Can affect any of them.
Why is diagnosing vasculitis difficult?
The clinical manifestations are diverse and can affect any organ/system. Therefore pin pointing this is tricky.
Primary vasculitis
Results from an inflammatory response that targets the vessel walls- no known cause.
Sometimes this can be autoimmune.
Secondary vasculitis
May be triggered by an infection, a drug, a toxin or may occur as part of another inflammatory disorder or cancer.
Pathogenesis of vasculitis
Activated dendritic cells release inflammatory mediators that promote the activation of T cells and cause inflammation, granuloma formation and macrophage activation.
Activated macrophages cause progressive endothelial damage, disruption of the internal elastic lamina and intimal hyperplasia.
Classification of vasculitis
International Chapel Hill consensus classification system
Could be large vessel e.g. Giant cell arteritis and Takayasu
Could be medium vessel e.g. Polyarteritis nodosa and Kawasaki disease
Or small vessel which are made up of immune complex associated vasculitis or ANCA associated vasculitis.
Large vessel vasculitis
Primary vasculitis causing chronic granulomatous formation primarily affecting the aorta and its major branches.
Categories of large cell vasculitis
Giant cell arteritis and takayasu arteritis.
Takayasu arteritis aetiology
Generally affects people of less than 40 years old and is much commoner in females. More common in Asian populations.
Giant cell arteritis aetiology
Generally affects those over 50.
Similarities between Takayasu arteritis and giant cell arteritis
They are both characterised by granulomatous infiltration in the walls of large vessels.
Presenting features of large cell vasculitis
Bruit- most commonly in the carotid artery
Blood pressure difference in the extremities
Claudication
Vessel tenderness or carotodynia (unilateral tenderness of the carotid artery)
Hypertension
Temporal arteritis association with other diseases
Associated strongly with polymyalgia rheumatica. About 50% of people with GCA have PMR.
Temporal arteritis symptoms
Unilateral headache Scalp tenderness Jaw claudication Temporal arteries may be prominent with reduced pulsation. Risk of blindness.
Investigations into large cell vasculitis
Inflammatory markers (ESR, CRP, Plasma viscocity) usually raised.
If there are symptoms of temporal arteritis- then temporal artery biopsy is needed.
Imaging such as MRI and pet scan may show vessel wall thickening/stenosis/aneurysm
Treatment of large cell vasculitis
Steroids- 40-60mg of prednisolone
Steroid sparing agents such as methotrexate could be considered.
Kawasaki disease aetiology
Seen in children- usually under 5 years.
Where does Kawasaki’s disease affect?
Can affect many vessels but predominantly the coronary arteries. This is where aneurysms can develop.
What is polyarteritis nodosa
Necrotising inflammatory lesions that arteries at vessel bifurcations resulting in microaneurysmal formations and aneurysm formations.
What is polyarteritis nodosa associated with?
Hepatitis B. It can also affect the skin, gut and kidneys.
Divisions in small vessel vasculitis
ANCA positive and ANCA negative.
New name for Wegners granulomatosis
Granulomatosis with polyangitis
New name for Churg Strauss syndrome
Eosinophillic granulomatosis with polyangitis
Pathology of eosinophillic granulomatosis with polyangitis
Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels. Associated with asthma
Pathology of granulomatosis with polyangitis
Granulomatous inflammation of respiratory tract, small and medium vessels. Necrotising glomerulonephritis common.
Pathology of microscopic polyangitis
Necrotising vasculitis with few immune deposits. Necrotising glomerulonephritis very common
Aetiology of granulomatosis with polyangitis (GPA)
GPA is more common in individuals of northern European descent (approximately 90%)
It has a male-to-female ratio of 1.5:1
The onset of GPA may occur at any age, although typically presents at age 35-55 years.
Constitutional symptoms and arthralgia are common.
ENT symptoms of GPA
Sinusitis Nasal crusting Epistaxis Mouth ulcers Sensorineural deafness Otitis media and deafness “Saddle nose” due to cartilage ischaemia
Respiratory symptoms of GPA
Pulmonary infiltrates Cough Hemoptysis Diffuse alveolar hemorrhage Cavitating nodules on CXR
Cutaneous feature of GPA
Palpable purpura
Cutaneous ulcers
Renal features of GPA
Necrotising glomerulonephritis
Manifests as blood and protein in urine
Nervous features of GPA
Mononeuritis multiplex
Sensorimotor polyneuropathy
Cranial nerve palsies
Ocular features of GPA
Conjunctivitis Episcleritis Uveitis Optic nerve vasculitis Retinal artery occlusion Proptosis
Features of EGPA
The same as GPA however there is late onset asthma and a high eosinophil count.
What are ANCA’s?
Anti-neutrophil cytoplasmic antibodies- they are autoantibodies that attack the cytoplasm of neutrophillic granulocytes.
How is ANCA detected?
Immunoflourescence.
This can differentiate between pANCA and cANCA.
GPA is associated with which type of antibodies?
cANCA and PR3
EGPA is associated with which type of antibodies?
pANCA and MPO
Management of autoimmune vasculitis
Management depends on degree of involvement.
Localised/early systemic- methotrexate and steroids
Generalised/systemic- Cyclophosphamide + steroids (1st line)
Rituximab + Steroids (alternative)
Plasma exchange if creatinine > 500
Refractory- IV Immunoglobulins
Rituximab
Henoch Schonlein Purpura
Acute IgA mediated disorder.
Generalized vasculitis involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system (CNS)
Approximately 75% of cases occur in children aged 2-11 years; HSP is rare in infants
How does Henoch schonlein purpura develop?
More than 75% of patients have had preceding URTI, pharyngeal infection, or GI infection
Most common is group A streptococcus
Preceding illness usually predates HSP by 1-3 weeks
Presentation of HSP
Purpuric rash typically over buttocks and lower limbs Colicky abdominal pain Bloody diarrhoea Joint pain +/- swelling Renal involvement (50%)
Management of HSP
Usually self limiting
Symptoms tend to resolve within 8 weeks.
Relapses may occur for months to years.
Urinalysis needs to be performed to screen for renal involvement.
