Vasculitis Flashcards
Vasculitis
Inflammation of blood vessels
Vasculitis Pathogenesis
An inflammatory process causes transcription factor and collagen to be exposed.
Leads to the formation of a thrombosis.
The vessel wall becomes leaky.
Leading to aneurysm.
Fibrosis occurs due to fibrin deposition.
Thrombosis and fibrosis narrow the vessel.
Leading to Reduced Blood Flow.
Vasculitis Vessel Changes
Vessel Wall thickening.
Vessel Stenosis
Occlusion of vessels with subsequent infarction.
Large Vessel Vasculitis Types (2)
Giant cell arteritis
Takayasu Arteritis
Large Vessel Vasculitis Early Features
Malaise night Sweats Weight loss Arthralgia Fatigue Claudicant Symptoms
Large Vessel Vasculitis Late Features
Vascular Stenosis
Aneurysms
Reduced pulses and bruit.
Large Vessel Vasculitis possible signs
Reduced pulses
Carotid Bruit
Giant Cell Arteritis - Arteries affected (3)
Temporal Artery
Opthalmis Artery
Facial Arteries
- jaw claudication if in facial arteries
Giant Cell Arteritis Epidemiology
Most common in women >50
Giant Cell Arteritis Histology
Granulomas are present in the elastic lamina
Takayasu Arteritis - Arteries affected
Affects the arteries off off the aortic arch
Takayasu Arteritis Epidemiology
Most common in Asian Women <40
Large Vessel Vasculitis Treatment
Treatment with 40-60mg Prednisolone
-Gradually reduced
Medium Vessel Vasculitis Types (3)
Kawasaki Disease
Polyarteritis Nodosa
Buerger’s Disease
Kawasaki Disease Features
Conjunctivitis Rash over body Adenopathy Strawberry Tongue Hands and feet swelling Fever
Kawasaki Disease Treatment
Self-limiting.
Can be treated with IV Ig and Aspirin
Polyarteritis Nodosa Pathology
Immune cells directly attack the endothelial cells.
Causes transmural necrosis. Leaves vessels prone to aneurysm.
Polyarthritis Nodosa Investigations
Angiogram
-‘string of beads’
Polyarthritis Nodosa Features
Can affect renal. lung and GI
- If renal, can lead to hypertension
- If GI, can lead to abdominal pain and GI bleeding
Buerger’s Disease
Affecting vessels in fingers and toes
Buerger’s Disease Risk Factor
Tobacco Use
Buerger’s Disease Features
Blood clots occur.
Cause ulceration, auto-amputation and necrosis
Small Vessel Vasculitis Types (4)
- Granulomatosis with Polyangiitis (GPA)
- Microscopic Polyangiitis (MPA)
- Eosinophilic granulomatosis with polyangiitis (EGPA)
- Henloch Schonlein Purpur
Granulomatosis with Polyangiitis (GPA) Epidemiology
More common in men
Typical age 35-55
Granulomatosis with Polyangiitis (GPA) Pathology
Granulomatous Inflammation of respiratory tract, small and medium vessels.
Granulomatosis with Polyangiitis (GPA) Features
Affects nasopharynx, lungs and kidneys Sinusitis Bloody mucus Saddle Nose Deformities Difficulty Breathing Relapse is common
Granulomatosis with Polyangiitis (GPA) Investigations
cANCA +ve
anti-PR3
Granulomatosis with Polyangiitis (GPA) Treatment
ANCA +ve vasculitis treated with IV steroids and cyclophosphamide
Microscopic Polyangiitis (MPA)
necrotising vasculitis with few immune deposits.
Microscopic Polyangiitis (MPA) Features
Doesn’t affect nasopharynx
No granulomas present
Can cause glomerulonephritis
Microscopic Polyangiitis (MPA) Investigations
pANCA +ve
High eosinophil count.
Eosinophilic granulomatosis with polyangiitis (EGPA)
Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels.
Eosinophilic granulomatosis with polyangiitis (EGPA) Affected Sites
Sinuses Lungs GI Kidneys Skin nerve Heart
Eosinophilic granulomatosis with polyangiitis (EGPA) Features
Granulomas are present
Associated with asthma
Eosinophilic granulomatosis with polyangiitis (EGPA) Investigations
Anti-MPO
anti-PR3
Increased eosinophils
No ANCA
Henloch Schonlein Purpura
Non-ANCA Vasculitis
Acute IgA mediated disorder
Often past history of URTI
Henloch Schonlein Purpura Pathology
IgA targets endothelial cells via molecular mimicry
Henloch Schonlein Purpur Features
palpable purpura due to fibrosis occurring in affected vessel
- over buttocks and lower legs
- abdominal pain
- vomiting
- joint pain
Can occur in GI tract and kidneys
GI- Haematuria
Renal- IgA Nephropathy
Henloch Schonlein Purpura Treatment
Can spontaneously resolve
Self limiting
Does not require treatment