Vasculitis Flashcards
What is vasculitis?
Inflammation of blood vessels, often with ishemia, necrosis and organ inflammation
What blood vessels can vasculitis affect?
Arteries, arterioles, vein, venules or capillaties
What is primary vasculitis?
Results from an inflammatory response that targets vessel walls with no known cause. Commonly autoimmune
What is secondary vasculitis?
Triggered by an infection, drug (e.g. cocaine) or a toxin that may occur as part of another inflammatory disorder or cancer
What will occur at a cellualr level in vasculitis?
Release of inflammatory cytokines activating T cells and vascular inflammation. This promotes granuloma formation and macrophage activation.
This will lead to inflammation, endothelial damage, disruption of elastic lamina and intimal hyperperplasia
What are examples of large-vessel vsaculitis?
Takayasu arteritiis
Giant Cell arteritis
What are examples of medium-vessel vasculitis?
Polyarteritis nodosa
Kawasaki disease
What are ANCA-positive small vessel vsaculitis?
Microscopic polyangitis
Granulomatosis with polyangitis (wegner’s)
Esoinophilic granulomatosis with polyangitis (churg-strauss)
What will churg strauss also present with along with vasculitis?
Rhinorea, asthma and eosinophilia
What non-ANCA small vessel vasculitis is Ig-A dominant?
Henoch-Sschnelin purpura
What are non-ANCA, immune complex small vessel vasculitis?
Cryoglubinemic vasculitis IgA vasculitis (henoch-Shconlein)
What non-ANCA small vessel vasculitis has high serum cryoglobulin?
Cryglobulinemia
What systemic symptoms can present with vasculitis?
Fever
Malaise
Weight loss
Fatigue
Who will TA affect?
Under 40
Females
Asian population
Who wil GCA affect?
Over 50
What are the presenting features of large cell vasculitis?
Bruit - most commonly around carotid artery
BP difference in extremities
Claudication
What muscle disease does GCA have an association with?
Polymyalgia Rheumatica
What are the symptoms of GCA?
Unilateral temporal headache
Scalp tenderness
Jaw claudication
What occular symptom can GCA present with?
Blindness due to ischaemia of optic nerve and opthalmic artery
What are the investigations for GCA?
ESR, PV and CRP
Temporal artery biopsy
MR anigiogram or PET CT
How is GCA managed?
40-60mg prednisolone
Higher dose if visual loss
What is the pathology of GPA?
Granulomatous inflammation of resp tract, small and medium vessels
Necrotising glomerulonephritis
What is the pathology of EGPA?
Eosinophilic granulomatous inflammation of resp tract, small and medium vessels
Associated with asthma
What is the pathology of MPA?
Necrotising vasculitis with few immune deposits
Necrotising glomerulonephritis very common
Who is likey to get GPA?
Slightly more common in males
35-55 years
Constitutional symptoms and arthralgia are common
What is the classification of GPA?
Nasal or oral inflammation
Abnormal chest radiograph - nodules, fixed infiltrates or cavities
Urinary sediment - microhaematuria or red cell casts
Granulomatous inflammation on biopsy
What ENT features can GPA show?
Sinusitis Nasal crusting Epistaxis Mouth ulcers Saddle nose
What resp features can GPA show?
Pulmonary infiltrates Cough Heamoptysis Diffuse alveolar haemorrhage CAvitating nodules
What cutaneous features can GPA show?
Palpable purpura
Cutaneous ulcers
What renal features can GPA show?
Necrotising golemrulonephritis
What nervous system features can GPA show?
Mononeuritis multiplex
Sensorimotor polyneuropathy
Cranial nerve palsies
What occular features can GPA show?
Conjunctivitis Episcleritis Uveitis Optic nerve vasculitis Retinal artery occlusion
What is the main difference between GPA and EGPA?
EGPA will occur with late onset asthma and a high eosinophil count
What is the critera for EGPA?
Ashtma
Eosinophilia of more than 10% in peripheral blood
Paranasal sinusitis
Pulmonary infiltrates
Histological proof of vasculitis with extravascular eosinophils
Mononeuritis multiplex or polyneuropathy
What condition will have c-ANCA and PR3 antibodies?
GPA
What condition will have p-ANCA MPO antibodies?
MPA and EGPA
How is anca associated vasculitis managed?
Cyclophosphamide
What is henoch-schonlein purpura (HSP)?
Acute IgA mediated disorder that causes generalised vasculitis
What can HSP affect?
Small vessels of skin GI trct Kidneys Joints RARE: lungs and CNS
Who is likely to get HSP?
Children aged 2-11
What will usually precede HSP?
Preceding URTI, pharyngeal infection or GI infection
Commonly group A streptococci
How will HSP present?
Purpuric rash over buttocks and lower limbs Colickly abdo pain Bloody diarrhoea Joint pain +/- swelling Renal involvement in 50%
How is HSP managed?
Usually self-limiting
Symptoms tend ot reslove within 8 weeis
Essential to perform urinalysis to screen for renal involvement
