Vasculitis Flashcards
Vasculitis
Inflammation of a blood vessel
Broken down into size of arteries
Antineutrophil cytoplasmic antibody-associated vasculitis (AAV)
life-threatening disorder -inflammatory infiltration of small blood vessels, fibrinoid necrosis and the presence of circulating antibodies to antineutrophil cytoplasmic antibody (ANCA)
combined incidence is about 10–15/1 000 000 two main
subtypes are recognised:
◦ (MPA) Microscopic polyangiitis is a necrotising small-vessel vasculitis found with rapidly progressive glomerulonephritis (kidney) often with alveolar haemorrhage (lung). Skin and gastrointestinal involvement is common and other features include neuropathy (15%) and pleural effusions (15%). Usually myeloperoxidase (MPO) antibody-positive
◦ (GPA) Granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) -granuloma formation, mainly affecting the nasal passages, airways and kidney. Some present with glomerulonephritis. Usually see nose and sinus disease (epistaxis, nasal crusting and sinusitis) Deafness may be a feature. Usually proteinase-3 (PR3) antibody-positive (ELISA).
Wegener’s term not used as much as he was a NAZI
WHAT TO KNOW:
SMALL VESSEL VASCULITUS IS ASSOCIATED WITH ANCA ANTIBODY
PICKED UP IN SERUM
PAIN IN VASCULITIS DUE TO NECROTIC BLOOD VESSELS AND TISSUE DISTAL TO INVOLVED BLOOD VESSEL
Investigations for Antineutrophil cytoplasmic antibody-associated vasculitis (AAV)
WCC, CRP, ESR and PR3 high
Imaging of the upper airways or chest with CT/MRI can be useful
biopsy of the kidney or lesions in the sinuses and upper airways if possible- to rule out cancer
Management:
◦ high-dose glucocorticoids
◦ intravenous cyclophosphamide- can cause infertility, Can cause irritability in bladder
◦ maintance with glucocorticoids azathioprine, methotrexate or MMF
◦ rituximab
AAV has a tendency to relapse and patients must be followed
IF THERE IS A PATTERN OF DISEASE WITH MORE THAN ONE ORGAN DISORDER, IS IT CONNECTIVE TISSUE RELATEDOR A VASCULITIS
THESE CONDITIONS HAVE VERY POOR PROGNOSIS IF UNTREATED
Takayasu arteritis
Skipped over in lectures
Larger vessel arteritis
Doesn’t tend to be associated with ANCA
As much as we need to know ^^
Added detail but not needed to know on card
affects the aorta, its major branches and occasionally the pulmonary arteries
typical age at onset is 25–30 years8 : 1 female-to-male ratio
most common in Asia.
granulomatous inflammation of the vessel wall, leading to occlusion or weakening of the vessel wall.
Clinical features: claudication, fever, arthralgia and weight loss
Clinical examination may reveal loss of pulses, bruits, hypertension and aortic incompetence
Investigation will identify an acute phase response and normocytic, normochromic anaemia
diagnosis is based on angiography, which reveals coarctation, occlusion and aneurysmal dilatation
treatment is with high-dose glucocorticoids and immunosuppressants, as described for ANCA-associated vasculitis
the 5-year survival is 83%
Kawasaki disease
Very rare
Learn more about in paeds
vasculitis that mostly involves the coronary vessels
It occurs mainly in Japan and other Asian countries
clinical features
◦ acute systemic disorder, usually affecting children under 5 years.
◦ fever, generalised rash, including palms and soles, inflamed oral mucosa and conjunctival injection
◦ cause is unknown but is thought to be an abnormal immune response to an infectious trigger
◦ Cardiovascular complications include coronary arteritis, leading to myocardial infarction, transient coronary dilatation, myocarditis, pericarditis, peripheral vascular insufficiency and gangrene
treatment is with aspirin (5 mg/kg daily for 14 days) and IVIg (400 mg/kg daily for 4 days)
Polyarteritis nodosa
Small vessel arteritis- ANCA negative
incidence between the ages of 40 and 50
male-to-female ratio of 2 : 1
annual incidence is about 2/1 000 000
Hepatitis B is an important risk factor
Clinical features include fever, myalgia, arthralgia and weight loss, and multisystem disease.
The most common skin lesions are
◦ palpable purpura
◦ Ulceration
◦ Infarction
◦ livedo reticularis
necrotising inflammation and vessel occlusion, and in 70% of patients arteritis of the
vasa nervorum leads to neuropathy
Severe hypertension and/or renal impairment may occur due to multiple renal infarctions but glomerulonephritis is rare (in contrast to microscopic polyangiitis)
The diagnosis is confirmed by angiography, which shows multiple aneurysms and smooth narrowing of mesenteric, hepatic or renal systems, or by muscle or sural nerve biopsy
Treatment is with high-dose glucocorticoids and immunosuppressants, as described for ANCA-associated vasculitis
Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome)
small-vessel vasculitis
incidence of about 1–3 per 1 000 000
associated with eosinophilia
may have a prodromal period for many years, characterised by allergic rhinitis, nasal polyposis and late-onset asthma
typical acute presentation is with a triad of skin lesions (purpura or nodules), asymmetric mononeuritis multiplex and eosinophilia.
Pulmonary infiltrates and pleural or pericardial effusions
Up to 50% of patients have abdominal symptoms -mesenteric vasculitis
raised levels of ESR and CRP and an eosinophilia.
MPO or PR3 antiobies can be detected in up to 60% of cases
biopsy of an affected site reveals a small-vessel vasculitis with eosinophilic infiltration of the vessel wall
management is with high-dose glucocorticoids and cyclophosphamide, followed by maintenance therapy with low-dose glucocorticoids and azathioprine, methotrexate or MMF.
Henoch–Schönlein purpura
Self limiting, not associated with ANCA
‘small-vessel vasculitis caused by immune complex deposition after an infectious trigger
disease of children and young adults
clinical features:
◦ purpura over the buttocks and lower legs
◦ accompanied by abdominal pain
◦ gastrointestinal bleeding
◦ arthralgia
◦ nephritis can also occur up to 4 weeks after other symptoms.
investigations:
◦ biopsy of affected tissue shows a vasculitis with IgA deposits in the vessel wall.
usually self-limiting disorder that settles spontaneously without specific treatment
glucocorticoids and immunosuppressive therapy may be required in patients with more severe disease, particularly if renal disease
Behçet’s disease
Family routes to the silk route- dyes and silks Of Middle East to Europe. People from Turkey
Very deep oral ulcers, genital ulcers.
unknown aetiology
targets small arteries and venules
rare in Western Europe but more common in ‘Silk Route’ countries
association with HLA-B51
oral ulcers in all - usually deep and multiple, and last for 10–30 days.
genital ulcers are also a common problem, occurring in 60–80% of cases.
skin lesions
◦ erythema nodosum
◦ acneiform lesions
◦ migratory thrombophlebitis ◦ vasculitis also occur.
eye involvement is common
◦ anterior or posterior uveitis or retinal vasculitis.
neurological involvement occurs in 5%
recurrent thromboses may occur
renal involvement is extremely rare
diagnosis is primarily made on clinical grounds pathergy test- papule where needle pierced skin
Criteria for the diagnosis of Behçet’s disease
Recurrent oral ulceration: minor aphthous, major aphthous or herpetiform ulceration at least three times in 12 months plus two of the following:
recurrent genital ulceration
eye lesions: anterior uveitis, posterior uveitis, cells in vitreous on slit-lamp examination, retinal vasculitis
skin lesions: erythema nodosum, pseudofolliculitis, papulopustular lesions, acneiform nodules
positive pathergy test
REMEMBER RECURRENT ORAL ULCERATION
Treatment:
oral ulceration
◦ topical glucocorticoid preparations (soluble prednisolone mouthwashes, glucocorticoid pastes)
erythema nodosum
◦ Colchicine can be effective for skin and arthralgia
oral and genital ulceration
◦ Thalidomide is teratogenic and neurotoxic.
uveitis and neurological disease
◦ Glucocorticoids and immunosuppressants
Rare non Vasculitis conditions:
Relapsing polychondritis
rare inflammatory disease of cartilage
clinical presentation acute pain and swelling of one or both ear pinnae
around 30% of patients have coexisting autoimmune or connective tissue disease.
involvement of tracheobronchial cartilage leads to a hoarse voice, cough, stridor or expiratory wheeze.
other features: collapse of the bridge of the nose, scleritis, hearing loss and cardiac valve dysfunction.
cartilage biopsy shows an inflammatory infiltrate in the perichondrium. both ESR and CRP are high
pulmonary function tests as affects laryngotrachea
mild disease usually responds to low-dose glucocorticoids or NSAIDs
major tracheobronchial involvement requires high-dose glucocorticoids and immunosuppressants, as described for SLE.
Adult-onset Still’s disease
! A rare systemic auto-inflammatory disorder
◦ intermittent fever,
◦ rash
◦ arthralgia
◦ splenomegaly, hepatomegaly and lymphadenopathy may be present.
◦ acute phase response (ferritin very high)
◦ CCP, RF and ANA are negative
◦ Treatment:
● glucocorticoids
● methorexate, azathioprine or MMF
● anakinra
Summary of what we need to know year 1 Vasculitis
Understand the term vasculitis
Understand ANCA
Build on this when you move onto other modules
