Vasculitis Flashcards

1
Q

What is vasculitis?

A

inflammation of the blood vessels

small vessels, medium sized vessels or large vessels

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2
Q

Types of Vasculitis Affecting The Small Vessels

A

Henoch-Schonlein purpura

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)

Microscopic polyangiitis

Granulomatosis with polyangiitis (Wegener’s granulomatosis)

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3
Q

Types of Vasculitis Affecting The Medium Sized Vessels

A

Polyarteritis nodosa

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)

Kawasaki Disease

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4
Q

Types of Vasculitis Affecting The Large Vessels

A

Giant cell arteritis

Takayasu’s arteritis

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5
Q

Generic presentation of most types of vasculitis (7)

A

Joint and muscle pain

Anterior uveitis and scleritis

Peripheral neuropathy

Gastrointestinal disturbance (diarrhoea, abdominal pain and bleeding)

Renal impairment

Hypertension

Purpura. These are purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin.

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6
Q

Systemic manifestations in vasculitis (5)

A

Fatigue

Fever

Weight loss

Anorexia (loss of appetite)

Anaemia

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7
Q

Tests in vasculitis

A

CRP and ESR

Anti neutrophil cytoplasmic antibodies (ANCA)

p-ANCA (MPO antibodies): Microscopic polyangiitis and Churg-Strauss syndrome

c-ANCA (PR3 antibodies): Wegener’s granulomatosis

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8
Q

Management of vasculitis

A

depends on the type

Steroids
Immunosuppressants

Different management in HSP and Kawasaki disease is different

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9
Q

What is HSP?

A

IgA vasculitis

Presents with a purpuric rash affecting the lower limbs or buttocks in children

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10
Q

What is a common trigger for HSP?

A

Upper airway infection

Gastroenteritis

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11
Q

At what age does HSP most commonly occur?

A

Children under the age of 10

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12
Q

Why does rash occur in HSP?

A

Inflammation occurs due to immunoglobulin A

Leaking of blood from small blood vessels under the skin

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13
Q

What are the 4 classic features of HSP?

A

Purpura (100%)

Joint pain (75%)

Abdominal pain (50%)

Renal involvement - IgA nephritis (50%)

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14
Q

Management of HSP

A

Typically supportive

Simple analgesia, rest and proper hydration

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15
Q

Recovery in HSP

A

Abdominal pain usually settles within a few days

Kidney involvement - full recovery in 4-6 weeks

1/3 have recurrence in 6 months

1% end stage renal failure

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16
Q

What is Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)?

A

Small and medium vessel vasculitis

Associated with lung and skin problems

17
Q

What is the characteristic finding on FBC in Churg-Strauss?

A

Elevated eosinophil levels

18
Q

How does Eosinophilic Granulomatosis with Polyangiitis often present?

A

Severe asthma in late teenage years or adulthood

19
Q

What is Microscopic polyangiitis?

A

small vessel vasculitis

20
Q

What are the features of Microscopic polyangiitis?

A

Main feature - renal failure

Can also affect lungs - SOB, haemoptysis

21
Q

What is Granulomatosis with polyangiitis (Wegener’s granulomatosis)?

A

small vessel vasculitis

affects the respiratory tract and kidneys

22
Q

What symptoms occur within the nose in Granulomatosis with polyangiitis?

A

Nosebleeds, crusty nasal secretions

Saddle shaped nose due to a perforated nasal septum - causes a dip halfway down the nose

23
Q

What symptoms occur within the ears in Granulomatosis with polyangiitis?

A

Hearing loss

Tinitus

24
Q

What symptoms occur in the lungs in Granulomatosis with polyangiitis?

A

Cough

Wheeze

Haemoptysis

25
Q

What symptoms occur in the kidney in Granulomatosis with polyangiitis?

A

Rapidly progressing glomerulonephritis

26
Q

What is Polyarteritis Nodosa?

A

Medium vessel vasculitis

Associated with hepatitis B

Can also occur in hep c or HIV

27
Q

Where does polyarteritis nodosa affect?

A

Skin

Gastrointestinal tract

Kidneys

Heart

28
Q

What rash is associated with polyarteritis nodosa?

A

Livedo reticularis

mottled, purplish, lace like rash

29
Q

What is Kawasaki Disease?

A

Medium vessel vasculitis

Typically affects children under 5 years of age

30
Q

Clinical features of Kawasaki disease

A

Persistent high fever > 5 days

Erythematous rash

Bilateral conjunctivitis

Erythema and desquamation (skin peeling) of palms and soles

“Strawberry tongue” (red tongue with prominent papillae)

31
Q

What is a key complication of Kawasaki disease?

A

Coronary artery aneurysms

32
Q

Treatment of Kawasaki

A

Aspirin

IV immunoglobulins

33
Q

Takayasu’s arteritis (pulseless disease)

A

Large vessel vasculitis - can form aneuryms

Mainly affects the aorta

Also pulmonary arteries

34
Q

When/how does Takayasu’s arteritis present?

A

Usually before the age of 40 years

Presents with:

Non-specific systemic symptoms - fever, malaise and muscle aches

or

Arm claudication or syncope