Vasculitides Flashcards
(30 cards)
What is vasculitis?
In its strictest sense, the term vasculitis denotes inflammation of a blood vessel, which is characterised by the presence of an inflammatory infiltrate and destruction of the vessel wall. Commonly, however, vasculitis refers to the systemic vasculitides, which are autoimmune disorders characterised by inflammation of blood vessels. The systemic vasculitides are a diverse group of disorders that demonstrate a wide range of organ involvement and clinical severity.
! How are systemic primary vasculitides classified? (x2, x2, x6 and x1)
- LARGE: Giant cell arteritis (GCA), Takayasu’s arteritis (TA)
- MEDIUM: polyarteritis nodosa (PAN), Kawasaki’s disease (KD)
- SMALL: Churg-Strauss syndrome (CSS AKA eosinophilic granulomatosis with polyangiitis), microscopic polyangitis (MP), Henock-Schonlein purpura (HSP), Wegener’s granulomatosis (WG AKA granulomatosis with polyangiitis), mixed essential cryoglobulinaemia (MEC) and relapsing polychondritis (RP)
- VARIABLE VESSEL: Behcet’s disease
What is the aetiology of primary vasculitides?
Autoimmune disorder of unknown origin.
What are the associations of vasculitis? (x2)
PAN – Hep B. MEC – Hep C.
What is the aetiology of systemic secondary vasculitides? (x3)
Infections, malignancies, connective tissue disorders such as RA and SLE.
What is the pathophysiology of vasculitis? (x4 points)
- Autoimmune complex deposition in vessel walls that trigger classical complement activation and inflammation
- Some vasculitides such as WG, MP and CSS are not associated with this pathophysiology. Instead, autoantibodies are ANTI-NEUTROPHIL CYTOPLASMIC AUTOANTIBODIES (pANCA) which cause vascular damage indirectly by priming neutrophils to degranulate and produce free radicals
- Some have evidence of granulomatous inflammation (GCA, WG, CS and PAN) – aggregates of macrophages in chronic inflammation
- In both cases, necrosis of the vessel wall and RBC extravasation is characteristic of vasculitis
What is fibrinoid necrosis?
When antigen-antibody complexes are deposited in the walls of blood vessels.
What is the epidemiology of vasculitis: Age? Ethnicity?
Over 50. White ethnicity. Particular forms affect specific populations.
What is the onset of vasculitis?
Sub-acute onset with symptoms and signs of chronic inflammation.
Principle of differentiating between large, medium and small vessel vasculitides?
Large vasculitides have patterns resulting from the vessel affected. Medium and small vessel vasculitides are characterised by multi-organ involvement with less specific clinical features.
GENERAL: What are the signs and symptoms of vasculitis?
- Constitutional symptoms: fever, night sweats, malaise, weight loss
- SKIN: purpuric, maculopapular, livedo reticularis rashes (small vessel)
- JOINT: arthralgia, myalgia, usually before onset of more specific symptoms
- GI: abdominal pain from ischaemia in medium vessel vasculitides, haemorrhage from mucosal ulceration, diarrhoea
- KIDNEY: glomerulonephritis, renal failure (small vessel)
- LUNG: dyspnoea, cough, chest pain, haemoptysis (small vessel)
- CVS: pericarditis, coronary arteritis, myocarditis
- CNS: mononeuritis multiplex, infarctions, meningeal involvement
- EYES: retinal haemorrhage, cotton wool spots, vision loss
What is a purpuric rash?
Skin haemorrhages, more common at extremities.
What is livedo reticularis?
Small blood clots that develop inside the blood vessels of the skin. It causes the skin to take on a blotchy red or blue appearance.

What is mononeuritis multiplex?
Asymmetric, painful, sensory and motor peripheral neuropathic inflammation of two or more nerves in random areas of the body.
What are the signs and symptoms characteristic of specific subtypes of vasculitis: large vessel vasculitides?
- GCA: see notes below
- TA: aka aortic arch syndrome; head/neck pain, tenderness over aorta and its major branches, dizziness, fainting, asymmetrical brachial pulses, reduced peripheral pulses, hypertension
What are the signs and symptoms characteristic of specific subtypes of vasculitis: medium vessel vasculitides?
- PAN: microaneurysms, thrombosis, infarctions, hypertension and testicular pain.
- KD: age below 5 years, fever lasting over 5 days, fissured lips, red swollen palms and soles followed by desquamation, skin rash, inflamed oral cavity, conjunctival congestion, lymphadenopathy, coronary artery aneurysm
What are the signs and symptoms characteristic of specific subtypes of vasculitis: small vessel vasculitides?
- CSS: allergic rhinitis, sinusitis, asthma, otorrhoea, ear pain
- (HSP: purpura on leg and buttocks, gut symptoms, glomerulonephritis with IgA deposition)
- MP: non-specific
- WG: granulomatous vasculitis of upper and lower respiratory tract, nasal discharge, sinusitis, corneal thinning
- (RP: affecting cartilage of ear, nose and larynx causing inflammation, hoarse voice)
- (MEC: splenomegaly, cryoglobulins)
What are the investigations for vasculitis? (x5)
- BLOOD: high ESR (>100)/CRP, normocytic anaemia, thrombophilia, neutrophilia
- AUTOANTIBODIES: see next flashcard
- URINE: glomerulonephritis in small vessel vasculitis
- BIOPSY: best for diagnosis. If skin lesion is taken, subcutaneous tissue also taken as contains medium vessels. Direct immunofluorescence should also be performed as the pattern Ig deposition may provide clues to aetiology e.g., IgA deposition in HSP.
- ANGIOGRAPHY: beading, aneurysm, or smooth tapering vessel stenosis in medium and large vessel vasculitides
What are the autoantibodies in vasculitis? (x3)
- cytoplasmic-ANCA: anti-proteinase-3 associated with WG
- perinuclear-ANCA: anti-myeloperoxidase associated with MP, CSS (but also IBD, PBC and chronic active hepatitis)
- Cryoglobulins (immunoglobulins (IgG, IgM) and complement components that precipitate at temperatures below 37 degrees) in MEC
What is giant cell arteritis?
AKA temporal/cranial/granulomatous arteritis. Granulomatous inflammation of large arteries, particularly of the external carotid artery, most commonly the superficial temporal artery.
What is the anatomy of the external carotid artery?
Some Anatomists Like Freaking Out Poor Medical Students

What is the aetiology of giant cell arteritis? (x2)
Unknown. Genetic: HLA-DR4 and HLA-DRB1, and infective association (Mycoplasma pneumoniae, parvovirus B19, Chlamydia pneumoniae).
! What is the pathophysiology of giant cell arteritis?
- Granulomatous inflammation in large arteries with preferential involvement of ECA but can also affect aorta and its other major branches.
- Initial immune insult occurs from resident dendritic cells presenting antigen to CD4+ T cells which release IFN-gamma that stimulates macrophage infiltration
- Granulomas are composed of T cells, macrophages, and multinucleated giant cells (fused macrophages)
- In response to damage, artery releases growth and angiogenic factors that result in myofibroblast proliferation, neovascularisation and marked thickening of the intimal layer
- This intimal expansion leads to occlusion of the vessel lumen leading to tissue ischaemia which leads to clinical symptoms
What is the epidemiology of giant cell arteritis: Common? Age? Gender? Ethnicity?
Most common systemic vasculitis. Peak age 65-70 years. More common in females. Most common in white ethnicities.