Vasculitis Flashcards
(45 cards)
Define vasculitis?
Inflammation of blood vessels, often with ischaemia and necrosis, thrombosis and organ inflammation
Areas affected by vasculitis?
Any blood vessel, inc. capillaries
Define primary and secondary vasculitis?
Primary: results from an inflammatory response that targets the vessel walls and has an UNKNOWN CAUSE; it is sometimes autoimmune
Secondary: may be triggered by an infection, drug, toxin or it may occur as part of another inflammatory disorder of cancer, e.g: paraneoplastic vasculitis assoc. with cancer
Pathophysiology of vasculitis?
- Activated dendritic cells release cytokines that activate T cells and cause vascular inflammation
- T cells promote inflammation, GRANULOMA formation and macrophage activation
- Macrophages release mediators that cause progressive vascular inflammation, endothelial damage and intimal hyperplasia
Main classifications of vaculitis?
Large-vessel, inc. Takayasu arteritis and giant cell arteritis
Medium-vessel, inc. polyarteritis nodosa and Kawasaki disease
Small-vessel is split into two categories
PICTURE 1
2 categories of small vessel vasculitis?
ANCA-associated small-vessel vasculitis (AAV):
• Granulomatosis with polyangiitis (GPA)
• Eosinophilic granulomatosis with polyangiitis (EGPA)
• Microscopic polyangiitis (MPA)
Immune complex small-vessel vasculitis:
• Henoch-Schönlein (common)
• Others
Systemic symptoms common to all vasculitides?
Fever
Malaise
Fatigue
Weight loss
Two main causes of large vessel vasculitis and histopathology?
Takayasu arteritis (TA)
Giant Cell arteritis (GCA)
There is GRANULOMATOUS infiltration of the large vessel walls
Occurrence of takayasu arteritis?
Tends to affect those <40 YEARS
More common in FEMALES
It is much more prevalent in Asian populations
Occurrence of GCA?
Generally affects those > 50 YEARS
Typically causes temporal arteritis but the aorta and other large vessels may be affected
Signs on examination of large vessel vasculitis?
- Bruit (usually if carotid artery is affected)
- BP difference of extremities
- Claudication (in Takayasu, this occurs suspiciously in younger people)
- Carotydinia or vessel tenderness (painful to touch)
- Hypertension
Occurrence of temporal arteritis?
Assoc. with polymyalgia rheumatica
About 50% with GCA have PMR, and about 15% of individuals with PMR develop GCA
Classical symptoms of temporal arteritis?
- Unilateral temporal headache
- Scalp tenderness
- Jaw claudication
- Prominent temporal arteries with reduced pulsation
- Pain over temporal artery
Risks with temporal arteritis?
Of blindness due to ischaemia of the optic nerve
Ix of temporal arteritis?
Inflammatory markers (ESR, PV and CRP) are raised
Temporal artery biopsy shows granulomas; biopsy may be -ve as there are SKIP LESIONS
MR angiogram can show stenosis/thickening/aneurysm
PET CT can shows increased metabolic activity of inflammatory cells
Management of temporal arteritis?
If there are no visual problems, 40 mg prednisolone gradually reduced over 18 months to 2 years
If there are visual problems, 60 mg prednisolone gradually reduced over 18 months to 2 years
Occasionally, if the patient struggles with a reduced steroid dose, methotrexate/azathioprine may be considered
Types of medium vessel vasculitis?
Kawasaki disease
Polyarteritis nodosa
What is Kawasaki disease?
Seen in CHILDREN, usually < 5 years, often following an INFECTION
Causes vasculitis of various vessels but can affect CORONARY ARTERIES, leading to aneurysm development
Tends to be self-limiting
What is polyarteritis nodosa?
Characterised by necrotising inflammatory lesions that affect arteries at vessel bifurcations, leading to microaneurysm and aneurym formation, and often affecting skin, gut and kidneys
Assoc. with HEP B
What were the different types of small vessel vaculitis previously known as?
GPA was previously known as Wegener’s granulomatosis
EGPA was previously known as Churg-Strauss syndrome
Microscopic polyangiitis has not changed name
Pathology of GPA?
Granulomatous inflammation of respiratory tract, small and medium vessels
Necrotising GLOMERULONEPHRITIS common
Pathology of EGPA?
Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels; increased eosinophils in blood and tissues
Assoc. with asthma
Pathology of MPA?
Necrotising vasculitis with few immune deposits
Necrotising GLOMERULONEPHRITIS very common
Ecologic factors in development of AAV?
Bacteria - Staph. aureus carriage in the nose increases risk of GPA
Genetic factors
Environmental, e.g: silica
Drugs, e.g: hydralazine
