Vasculitis Flashcards
Definition
Inflammation of blood vessels
Different sizes of blood vessels can be affected
Pathogenesis
WBC or immune complexes are present in the vessel wall
This damages the structure of the vessel wall leading to narrowing which ultimately causes ischaemia and claudication
2 types of large vessel vasculitis
Giant cell arteritis (GCA) Takayasu arteritis (TA)
GCA - definition
Granulomatous vasculitis affecting large arteries. It primarily affects the branches of the external carotid artery
GCA - epidemiology
Over 50
More common in women
GCA - clinical features
Headaches affecting the temples
Scalp tenderness
Jaw claudication
Sudden changes in vision (blindness)
GCA - examination
bruit (due to turbulent blood flow)
Loss of pulse at temporal artery
GCA - investigations
Inflammatory markers - raised
Temporal artery biopsy
GCA - management
High dose steroids (40-60mg prednisolone)
If patient is suffering from loss in vision then use higher dose of steroid
TA - definition
Vasculitis affecting the large arteries - mainly the aorta and pulmonary artery
TA - epidemiology
Under 40
Commonly affects females
TA - clinical features
Initially non-specific: malaise, fatigue, weight loss, lightheaded, SOB, chest pain
Arm/leg pain when moving
TA - investigations
Inflammatory markers - raised
PET CT scanning
TA - management
High dose steroids (40-60mg prednisolone)
Immunosuppression
2 main types of ANCA +ve small vessel vasculitis
Granulomatosis with polyangiitis (GPA) - aka Wegners
Eosinophilic granulomatosis with polyangiitis (EGPA) - aka churg strauss
GPA - definition
Systemic small vessel vasculitis which involves the respiratory tract along with other organs
+ve ANCA
GPA - epidemiology
Commonly affects males
Age: 35-55
GPA - clinical features
ENT involvement
- hearing loss, nose bleeds, sinusitis, mouth ulcers, defects of cartilage in nose (saddle shaped nose)
Resp involvement
- cough, haemoptysis, round cavitiating granuloma
Skin involvement
- purpuric rash, on lower legs, palpable
Kidney involvement
GPA - investigations
ANCA testing cANCA - specific for GPA anti-PR3 - varies with disease activity Complement levels - decreased Do renal biopsy to check for renal involvement
GPA - management
Localised: methotrexate/azathioprine + steroids
Generalised: IV cyclophosphamide + IV steroids
EGPA - definition
Systemic small vessel vasculitis which involves the respiratory tract and other organs. Eosinophils are found in high levels
ANCA +ve
EGPA - epidemiology
Middle aged
EGPA - clinical features
LESS ENT INVOLVEMENT THAN GPA
late onset asthma
rhinitis
EGPA - investigations
Increased eosinophil count
Biopsy - proof of vasculitis with extravascular eosinophils
ANCA testing
pANCA
anti-MPO - varies with disease activity
Complement levels - decreased
Do renal biopsy to check for renal involvement
EGPA - management
IV cyclophosphamide + IV steroids
Non ANCA associated small vessel vasculitis (1)
Henoch- Schonlein Purpura (HSP)
HSP - definition
Acute IgA mediated disorder characterised by a generalised vasculitis associated with other body systems
HSP - epidemiology
Commonly affects children
HSP - clinical features
Hx of URTI provokes disease
Purpuric rash over buttocks and lower limbs
Joint pain
Vomiting, diarrhoea, abdo pain
HSP - causative organism
Usually group A strep
HSP - investigations
Urinalysis (check for renal involvement)
HSP - management
Self limiting
