Vasculitis Flashcards
What is vasculitis?
A group of inflammatory blood vessel disorders.
There is damage of the blood vessel walls with subsequent thrombosis, ischaemia, bleeding and/or aneurysm formation.
What classifications of vasculitis are there?
Small vessel vasculitis
Medium vessel vasculitis
Large vessel vasculitis
Examples of large vessel vasculitis.
Giant cell arteritis
Takayasu’s arteritis
Examples of medium vessel vasculitis.
Classical polyarteritis nodosa (PAN)
Kawasaki’s disease
Examples of small vessel vasculitis.
ANCA-associated;
Microscopic polyangiitis
Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis
ANCA-negative;
IgA vasculitis (Henoch-Schönlein)
Cutaneous leucocytoclastic vasculitis
Essential cryoglobulinaemia
What is Takayasu’s arteritis?
A large vessel vasculitis that is rare except in Japan.
It is known as the pulseless disease or aortic arch syndrome.
Occurs predominantly in females.
Age of 40 years with non-specific systemic symptoms, such as fever, malaise and muscle aches, or with more specific symptoms of arm claudication or syncope.
It is diagnosed using CT or MRI angiography. Doppler ultrasound of the carotids can be useful in detecting carotid disease.
Vasculitis of the aortic arch as well as other major arteries.
Corticosteroids only help the constitutional symptoms.
Heart failure and stroke will follow.
Difference between GCA and Takayasu’s.
What is polyarteritis nodosa.
A rare condition that usually occurs in middle-aged men.
Severe systemic manifestations that is occassionally associated with Hep B.
This suggests that it is secondary to deposition of immune complexes.
Causes renal impairment, strokes and MIs.
Associated with a rash called livedo reticularis.
Clinical features of Polyarteritis Nodosa.
Fever
Malaise
Weight loss
Fever
Mononeuritis multiplex
Abdominal pain mimicking acute cholecystitis, pancreatitis or appendicitis.
Haematuria and proteinuria
Coronary arteritis
Subcut haemorrhage
Investigations in polyarteritis nodosa.
Bloods - anaemia, leucocytosis and raised ESR
Biopsy showing fibrinoid necrosis of vessel walls with microaneurysm formation.
Angiography showing microaneurysms
USS or ECG.
Treatment of Polyarteritis Nodosa.
Control BP and refer.
Steroids for mild cases and steroid sparing agents if more severe.
Hep-B should be treated after initial treatment with steroids.
What is Kawasaki’s disease?
An acute systemic vasculitis involving medium-sized vessels.
It happens in children under 5 years old with an infective trigger.
Clinical features of Kawasaki’s.
Fever lasting longer than 5 days
Bilateral conjunctivitis
Dry red lips
Cervical lymphadenopathy
Erythematous rash
Strawberry tongue
Treatment of Kawasaki’s
Aspirin + IV immunoglobulins according to Zero To Finals
Coronary artery aneurysm is a key complication
General history to ask about vasculitis.
Consider age, gender and ethnicity.
Drug history
FH of vasculitis
Constitutional symptoms such as fever, weight loss, malaise, fatigue, diminshed appetite and sweats.
How to approach symptom and sign history in vasculitis.
Glove and sweater approach.
Explain the glove and sweater approach in vasculitis.
Ask about Raynaud’s
MSK - arhtralgia, myalgia, proximal muscle weakness
CNS/PNS
Nose bleeds
Heart/lung
GI
Renal
Limbs
CNS/PNS symptoms in vasculitis.
Headaches
Visual loss
Tinnitus
Stroke
Seizure
Encephalopathy
Mucous symptoms in vasculitis.
Nose bleeds
Crusts
Ulcers
Oral ulcers
Heart and lung symptoms in vasculitis.
Pericarditis
Cough
Chest pain
Haemoptysis
Dyspnoea
GI symptoms in vasculitis.
Abdo pain
Renal symptoms in vasculitis.
Haematuria
Proteinuria
Possible renal failure
Limbs symptoms in vasculitis.
Neuropathy
Digital ulcers
Ischaemia
Physical exam of vasculitis.
Vital signs like BP (hyper), and pulse
Skin exam
Neurologic exam
Ocular exam
Cardiopulmonary exam
Abdo exam
Skin examination findings in vasculitis.
Palpable purpura
Livedo reticularis
Nodules
Digital ulcers
Gangrene
Nail bed capillary changes
Neurologic examination findings in vasculitis.
Cranial nerve exam
Sensorimotor exam
Ocular examination findings in vasculitis.
Visual field defects
Scleritis
Uveitis
Episcleritis
Cardiopulmonary examination findings in vasculitis.
Crackles
Pleural rubs
Murmurs
Arrhythmias
Abdominal examination findings in vasculitis.
Tenderness
Organomegaly
Secondary causes of vasculitis.
INfections
Drugs
Malignancy
Connective tissue disease
Secondary is more common than primary
Investigations in vasculitis.
Urine dipstick +/- microscopy for GN
FBC, U&Es, LFTs, CRP, PV, ESR
ANA, ANCA, RF
C3, C4
Screen for Hep B, C, HIV and Cryoglobulins
Serum and urine protein electrophoresis
CK
Blood cultures
ECG
CXR
CT
MRI
Arteriography
CT-PET
Treatment of vasculitis.
Rule out infection.
1st line - Prednisolone: 1 mg/kg/day (maximum 60-80 mg/day) orally until symptoms resolve or for 1 month, then gradually taper over 6 months
Can also be given IV, nasal (for nasal symptoms) or inhaled for Churg-Strauss
Also give a bone protective agents.
2nd line - Try an antimetabolite like methotrexate or azathioprine. Can also try leflunomide, mycophenolate mofetil.
If refractory disease can try biologics.
In acute setting give cyclophosphamide.
Atrial myxomas can present with a vasculitis like syndrome.
How can this be excluded?
ECHO
Explain granulomatosis polyangiitis.
Wegener’s granulomatosis is a small vessel vasculitis. It affects the respiratory tract and kidneys.
Epistaxis and crusty nasal secretions
Hearing loss
Sinusitis
Saddle shaped nose
In the lungs it causes a cough, wheeze and haemoptysis.
A chest xray may show consolidation and it may be misdiagnosed as pneumonia.
In the kidneys it can cause a rapidly progressing glomerulonephritis.
What is microscopic polyangiitis?
Microscopic polyangiitis is a small vessel vasculitis.
The main feature of microscopic polyangiitis is renal failure. It can also affect the lungs causing shortness of breath and haemoptysis.
What is Churg-Strauss?
Eosinophilic granulomatosis with polyangiitis
It is most associated with lung and skin problems, but can affect other organs such as kidneys.
It often presents with severe asthma in late teenage years or adulthood. A characteristic finding is elevated eosinophil levels on the full blood count.
What is Henoch-Schönlein purpura?
IgA vasculitis that commonly presents with a purpuric rash affecting the lower limbs or buttocks in children. Inflammation occurs due to immunoglobulin A deposits in the blood vessels of affected organs such as the skin, kidneys and gastro-intestinal tract.
The condition is often triggered by an upper airway infection (e.g. tonsillitis) or a gastroenteritis. It is most common in children under the age of 10 years. The rash is caused by inflammation and leaking of blood from small blood vessels under the skin, forming purpura.
Four classical features of HSP.
The four classic features are
Purpura (100%)
Joint pain (75%)
Abdominal pain (50%)
Renal involvement (50%).
HSP affects the kidneys in about 50% of patients, causing an IgA nephritis.
Management of HSP
Management is typically supportive, such as simple analgesia, rest and proper hydration. The benefits of steroids are unclear.
The abdominal pain usually settles within a few days. Patients without kidney involvement can expect to fully recover within 4-6 weeks. A third of patients have a recurrence of the disease within 6 months. 1% of patients will go on to develop end stage renal failure.
