Multi-system Autoimmune Disease (Vasculitidies)

Question 1

What is Giant cell Arteritis

Answer 1

Giant cell arteritis is a systemic vasculitis of the medium and large arteries. It typically presents with symptoms affecting the temporal arteries and is also known as temporal arteritis.

Question 2

What is the main presenting symptom of Giant cell Arteritis

Answer 2

Persistent, severe head pain, usually in your temple area

Scalp tenderness

Jaw pain when you chew or open your mouth wide

Fever

Fatigue

Unintended weight loss

Vision loss or double vision, particularly in people who also have jaw pain

Sudden, permanent loss of vision in one eye

Question 3

What is the main complication of Giant cell Arteritis

Answer 3

The key complication of giant cell arteritis is vision loss. This is often irreversible. High dose steroids are used immediately once a diagnosis is suspected to prevent the development of progression of vision loss.

Question 4

What are the classifications of Giant cell arteritis

Answer 4

Three of the following (classic temporal arteritis)

• Age at onset ≥50 years
• New headache
• Temporal artery tenderness/reduced pulsation
• ESR≥50
• Abnormal temporal biopsy

Question 5

What are the Investigations for giant cell arteritis

Answer 5

Biopsy (gold standard for confirmation)
• Erythrocyte sedimentation rate.Commonly referred to as the sed rate, this test measures how quickly red blood cells fall to the bottom of a tube of blood. Red cells that drop rapidly might indicate inflammation in your body.
• C-reactive protein (CRP).This measures a substance your liver produces when inflammation is present.

Question 6

What is Imaging tests for giant cell arteritis

Answer 6

Doppler ultrasound.This test uses sound waves to produce images of blood flowing through your blood vessels.

Magnetic resonance angiography (MRA).This test combines anMRIwith the use of a contrast material that produces detailed images of your blood vessels.

Positron emission tomography (PET).. This test uses an intravenous tracer solution that contains a tiny amount of radioactive material.

APETscan can produce detailed images of your larger blood vessels and highlight areas of inflammation.

Question 7

What is the Treatment for giant cell arteritis

Answer 7

• Urgent initiation of high dose Prednisolone
• 40-60mg per day, gradually tapered
• PPI
• Bone protection
• Steroid sparing medication

Question 8

What are the vasculitides

Answer 8

The vasculitides are a heterogeneous group of conditions typified by their ability to cause vessel inflammation with or without necrosis. They present with a wide variety of signs and symptoms and, if left untreated, carry a significant burden of mortality and morbidity

Question 9

What are the vasculitides conditions

Answer 9

Granulomatosis with polyangiitis (Wegener’s) (62.5%)

Microscopic polyangiitis (16.7%)

Eosiniphilic granulomatosis with polyangiitis (20.8%)

Question 10

What is Granulomatosis with polyangiitis (Wegener’s)

Answer 10

Necrotising granulomatous inflammation
Usually involving the upper and lower respiratory tract
Hearing loss, sinusitis, hemoptysis
Necrotising glomerulonephritis is common
cANCA, anti PR3 antibodies

Question 11

What is Microscopic polyangiitis

Answer 11

Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels
Granulomatous inflammation is absent
Renal and pulmonary involvement common
pANCA, anti MPO antibodies

Question 12

What is Eosiniphilic granulomatosis with polyangiitis

Answer 12

Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract
Late onset asthma, nasal polyps and eosinophilia
Necrotising vasculitis predominantly affecting small to medium vessels
Neurological involvement
Cardiac, gastrointestinal – poor prognosis
40-60% anti MPO antibodies positive