Vasculitis Flashcards
State the 4 vasculitis that affect the small blood vessels
- Henoch-Shonlein purpura
- Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
- Microscopic polyangiitis
- Granulomatosis with polyangiitis (Wegener’s granulomatosis)
State the 3 vasculitis that afffect the medium sized vessels
- Polyarteritis nodosa
- Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
- Kawasaki disease
State the 2 vasculitis that affect the large blood vessels
- Takayasu’s arteritis
- Giant cell arteritis
There are some generic featurest that apply to most types of vasculitis; state these
- Pupura
- Joint & muscle pain
- Peripheral neuropathy
- Renal impairment
- GI disturbance (diarrhoea, abdo pain, bleeding)
- Anterior uveitis & slceritis
- Hypertension
Also systemic features…
- Fatigue
- Fever
- Weight loss
- Anorexia
- Anaemia
What is purpura?
Purple coloured, non-blanching spots caused by blood leakage from vessels under skin
What investigations would you do if you suspect vasculitis?
*Just asking for initial ones you would do to support diagnosis of vasculitis; in reality many more you would do based on symptoms etc…
- Inflammatory markers (ESR & CRP)
- ANCA (anti-neutrophil cytoplasmic antibodies)
- pANCA
- cANCA
What are the following also called:
- pANCA
- cANCA
- pANCA = anti-MPO
- cANCA= anti-PR3
What vasculitis are pANCA (anti-MPO) antibodies found in?
- Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
- Microscopic polyangiitis
What vasculitis are cANCA (anti-PR3) antibodies found in?
Granulomatosis with polyangiitis (Wegener’s granulomatosis)
Managemennt of vasculitis depends on time and any suspected cases should be referred to specialist, usually rheumatologist, for diagnosis & treatment. Discuss the general principles of management of vasculitis
Treatment usually involves combination of steroids & immunosupressants
- Steroids can be administered to target area:
- Oral
- Intravenous
- Nasal sprays
- Inhaled
- Immunosupressants
- Cyclophosphamide
- Methotrexate
- Azathioprine
- Rituximab (and other monoclonal antibodies)
*NOTE: management of HSP and Kawasaki disease (types mainly affecting children) is different!!!!
For Henoch-Schonlein purpura, state:
- How it presents
- What age range it usually affects
- Pathophysiology
- Common triggers
- Management
- Usually presents with purpuric rash affecting lower limbs or buttocks in children. Four classical features:
- Purpura (100%)
- Joint pain (75%)
- Abdo pain (50%)
- Renal involvement (50%) *IgA nephritis
- <10yrs
- Immunoglobulin A deposits in blood vessels of affected organs
- Triggers:
- URT infection e.g. tonsillitis
- Gastroenteritis
- Management is supportive:
- Simple analgesia
- Rest
- Hydration
Discuss the prognosis of Henloch-Schonlein purpura
*HINT: consider recovery time, recurrence & progression
- Pts without kideny involvement can expect to make full recovery in 4- weeks
- Abdo pain usually settles in few days
- 1/3 pts have recurrence in 6 months
- 1% go on to develop ESRD
For Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome) discuss:
- Which two body systems it most commonly affects
- How it commonly presents
- Characteristic finding on FBC
- Lungs & skin (but can affect other organs e.g. kidneys)
- Often presents as severe asthma in late teens or adulthood
- Elevated eosinophils on FBC
For microscopic polyangiitis, discuss:
- What the main feature is
- What other systems it can affect
- Main features is renal failure
- Can also affect lungs causing SOB & haemoptysis
For Granulomatosis with Polyangiitis (Wegener’s syndrome), discuss:
- Which systems it usually affects
- How it commonly presents (include symptoms & signs)
- What may you see on CXR
- Commonly affects respiratory tract & kidneys
- Typical presentation:
- Epistaxis
- Crusty nasal secretions
- Hearing loss
- Sinusitis
- Saddle shaped nose (due to perforated nasal septum)
- Cough
- Wheeze
- Haemoptysis
- Rapidly progressive glomerulonephritis
- May see consolidation on CXR hence can be misdiagnosed as pneumonia
For polyarteritis nodosa, discuss:
- What infections it is associated with
- Which systems it commonly affects
- What rash it is associated with
- Most associated with Hep B but can also be associated with Hep C, HIV and without a clear cause
- Medium sized vessels in skin, GI tract, kidneys & heart (can cause renal impairment, strokes, MI)
- Livedo retiularis (mottled, purplish, lace-like rash)
For Kawasaki disease, discuss:
- Age range it usually affects
- Cause
- Clinical features
- Key complication
- Treatment
- Children <5yrs
- No clear cause
- Clinical features:
- Persistent high fever >5 days
- Erythematous rash
- Bilateral conjunctivitis
- Erythema & desquamation (skin peeling) of palms & soles
- Strawberry tongue (red tongue with strawberry papillae)
- Key complication= coronary artery aneurysms
- Treatment:
- Aspirin
- IV immunoglobulins
For Takayasu’s arteritis, discuss:
- Which vessels it mainly affects
- It’s alternative name and why it has this name
- Age of presentation
- Typical presentation
- Diagnosis
- Mainly affects aorta & it’s branches- also affects pulmonary arteries
- “Pulseless disease” as vessels and their branches can swell to form aneuryss or become narrowed and blocked- hence pulseless
- Before 40yrs
- Presentation:
- Non-specific: fever, malaise, muscle aches
- Specific: arm claudication, syncope
- Diagnosed using CT or MRI angiography. Doppler US of carotids can help to detect carotid disease.
