Vasculitis Flashcards

1
Q

What is vasculitis?

A

A group of inflammatory blood vessel disorders.

There is damage of the blood vessel walls with subsequent thrombosis, ischaemia, bleeding and/or aneurysm formation.

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2
Q

What classifications of vasculitis are there?

A

Small vessel vasculitis

Medium vessel vasculitis

Large vessel vasculitis

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3
Q

Examples of large vessel vasculitis.

A

Giant cell arteritis

Takayasu’s arteritis

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4
Q

Examples of medium vessel vasculitis.

A

Classical polyarteritis nodosa (PAN)

Kawasaki’s disease

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5
Q

Examples of small vessel vasculitis.

A

ANCA-associated;
Microscopic polyangiitis
Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis

ANCA-negative;
IgA vasculitis (Henoch-Schönlein)
Cutaneous leucocytoclastic vasculitis
Essential cryoglobulinaemia

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6
Q

What is Takayasu’s arteritis?

A

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.

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7
Q

Difference between GCA and Takayasu’s.

A
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8
Q

What is polyarteritis nodosa.

A

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.

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9
Q

Clinical features of Polyarteritis Nodosa.

A

Fever

Malaise

Weight loss

Fever

Mononeuritis multiplex

Abdominal pain mimicking acute cholecystitis, pancreatitis or appendicitis.

Haematuria and proteinuria

Coronary arteritis

Subcut haemorrhage

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10
Q

Investigations in polyarteritis nodosa.

A

Bloods - anaemia, leucocytosis and raised ESR

Biopsy showing fibrinoid necrosis of vessel walls with microaneurysm formation.

Angiography showing microaneurysms

USS or ECG.

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11
Q

Treatment of Polyarteritis Nodosa.

A

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.

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12
Q

What is Kawasaki’s disease?

A

An acute systemic vasculitis involving medium-sized vessels.

It happens in children under 5 years old with an infective trigger.

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13
Q

Clinical features of Kawasaki’s.

A

Fever lasting longer than 5 days

Bilateral conjunctivitis

Dry red lips

Cervical lymphadenopathy

Erythematous rash

Strawberry tongue

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14
Q

Treatment of Kawasaki’s

A

Aspirin + IV immunoglobulins according to Zero To Finals

Coronary artery aneurysm is a key complication

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15
Q

General history to ask about vasculitis.

A

Consider age, gender and ethnicity.

Drug history

FH of vasculitis

Constitutional symptoms such as fever, weight loss, malaise, fatigue, diminshed appetite and sweats.

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16
Q

How to approach symptom and sign history in vasculitis.

A

Glove and sweater approach.

17
Q

Explain the glove and sweater approach in vasculitis.

A

Ask about Raynaud’s

MSK - arhtralgia, myalgia, proximal muscle weakness

CNS/PNS

Nose bleeds

Heart/lung

GI

Renal

Limbs

18
Q

CNS/PNS symptoms in vasculitis.

A

Headaches

Visual loss

Tinnitus

Stroke

Seizure

Encephalopathy

19
Q

Mucous symptoms in vasculitis.

A

Nose bleeds

Crusts

Ulcers

Oral ulcers

20
Q

Heart and lung symptoms in vasculitis.

A

Pericarditis

Cough

Chest pain

Haemoptysis

Dyspnoea

21
Q

GI symptoms in vasculitis.

A

Abdo pain

22
Q

Renal symptoms in vasculitis.

A

Haematuria

Proteinuria

Possible renal failure

23
Q

Limbs symptoms in vasculitis.

A

Neuropathy

Digital ulcers

Ischaemia

24
Q

Physical exam of vasculitis.

A

Vital signs like BP (hyper), and pulse

Skin exam

Neurologic exam

Ocular exam

Cardiopulmonary exam

Abdo exam

25
Q

Skin examination findings in vasculitis.

A

Palpable purpura

Livedo reticularis

Nodules

Digital ulcers

Gangrene

Nail bed capillary changes

26
Q

Neurologic examination findings in vasculitis.

A

Cranial nerve exam

Sensorimotor exam

27
Q

Ocular examination findings in vasculitis.

A

Visual field defects

Scleritis

Uveitis

Episcleritis

28
Q

Cardiopulmonary examination findings in vasculitis.

A

Crackles

Pleural rubs

Murmurs

Arrhythmias

29
Q

Abdominal examination findings in vasculitis.

A

Tenderness

Organomegaly

30
Q

Secondary causes of vasculitis.

A

INfections

Drugs

Malignancy

Connective tissue disease

Secondary is more common than primary

31
Q

Investigations in vasculitis.

A

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

32
Q

Treatment of vasculitis.

A

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.

33
Q

Atrial myxomas can present with a vasculitis like syndrome.

How can this be excluded?

A

ECHO

34
Q

Explain granulomatosis polyangiitis.

A

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.

35
Q

What is microscopic polyangiitis?

A

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.

36
Q

What is Churg-Strauss?

A

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.

37
Q

What is Henoch-Schönlein purpura?

A

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.

38
Q

Four classical features of HSP.

A

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.

39
Q

Management of HSP

A

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.