Vasculitis - Primary Vasculitis Flashcards
(33 cards)
List 2 types of large vessel vasculitis.
Temporal arteritis
Takayasu’s disease
What sort of patients are affected by temporal arteritis?
What about Takayasu’s disease?
Temporal arteritis: elderly women
Takayasu’s: young women
Describe the clinical features of temporal arteritis. (6)
General inflammation Thickened, visible temporal a. Tenderness in temples/scalp Headache Fatigue Jaw claudication
What specific test would you do for temporal arteritis?
Describe the results. (3)
Biopsy
Features:
- Small lumen of artery
- Thickened vessel walls
- Inflammatory infiltrate
List 7 complications of temporal arteritis.
Affects nearby vessels (e.g. ophthalmic a., internal carotid a.)
Amaurosis fugax (sudden, painless blindness)
Tongue paraplesia
Sensorineural deafness
Stroke
Involvement of aorta
Coronary ischaemia
List 1 condition which is associated with temporal arteritis.
Polymyalgia rheumatica
How would you manage temporal arteritis?
Steroids (oral prednisolone)
Describe the clinical features of Takayasu’s disease. (7)
SYMPTOMS: Syncope Facial muscle paralysis Transient blindness Claudication Systemic features
SIGNS:
Absent pulses
BP variable between limbs
Which investigations would you do for Takayasu’s disease? (2)
Angiography
MRI
Define Takayasu’s disease.
What are 2 other names for this condition?
Pulseless disease
Aortic arch syndrome
Def.: “Progressive occlusion of the arteries arising from the arch of the aorta, resulting in absent pulses in arms/neck”
How would you treat Takayasu’s disease? (4)
Steroids (oral prednisolone)
BP control
Methotrexate/cyclophosphamide
Angioplasty/stenting (for critical stenosis)
List 2 examples of medium vessel vasculitis.
Polyarteritis nodosa
Kawasaki’s disease
Define polyarteritis nodosa.
Connective tissue disorder of unknown cause in which there is patchy inflammation of the artery walls
Describe the clinical features of polyarteritis nodosa. (3)
Systemic features, e.g.
- Fever
- Weight loss
- Night sweats
Organ infarction
Organ-specific symptoms
Describe the consequences of polyarteritis nodosa in:
a) Kidneys (3)
b) Gut (1)
c) CV system (3)
d) MSK system (2)
e) Skin (2)
KIDNEYS:
Renal artery stenosis
Renal aneurysm
Hypertension
GUT:
Bowel infarction
CV SYSTEM:
Stroke
MI
Hypertension
MSK SYSTEM:
Arthritis
Critical limb ischaemia
SKIN:
Gangrene
Purpura
Define Kawasaki disease.
Condition of unknown cause affecting children <5 yo, characterised by fever, conjunctivitis, sore throat, generalised rash and reddening of palms/soles
Describe the clinical features of Kawasaki disease. (8)
Large, pulsating aneurysms in several arteries
High fever
Other features, e.g.
- Mucositis
- Conjunctivitis
- Strawberry tongue
- Nail changes
- Palmar erythema
- Desquamation
Describe the natural progression of Kawasaki disease.
HINT: there are 2 phases.
ACUTE PHASE (10 days)
- Good prognosis
- Presentation: signs of infection
SUBACUTE PHASE (10+ days)
- Worse prognosis
- Presentation: asymptomatic aneurysms; often only detected when they rupture and baby dies
List 3 examples of immune complex mediated small vessel vasculitis.
Goodpasture’s syndrome
Cryoglobulinaemia
Henoch-Schonlein Purpura (HSP)
Define Henoch-Schonlein purpura (HSP).
Common, recurrent form of purpura usually found in young children, characterised by red weals and a purple rash on the buttocks/lower legs due to bleeding into the skin from inflamed capillaries
Describe the clinical features of HSP. (5)
What is the classical HSP triad?
HSP TRIAD:
Palpable purpura
Abdominal pain
Arthritis
Distribution of purpura:
- Extensor surfaces
- Starts peripherally, spreads proximally
Describe the pathophysiology of HSP. (2)
- Unrelated infection stimulates formation of IgA deposits in small blood vessels
- This causes small vessel vasculitis
List 7 complications of HSP.
KIDNEYS:
Nephritic syndrome
BOWEL:
Intussusception
Bowel infarction
Purpura (doughnut sign on colonoscopy)
EMOTIONS:
Pain and misery
TESTES:
Testes regression
Infarction
How would you manage HSP? (1)
Supportive treatment
