Large vessel vasculitides

Question 1

TAKAYASU ARTERITIS

Answer 1

General features

• In females; age less than 40 years
• Pathomechanism: T-cell-mediated immune response to vessel wall antigen(s)

Morphology

• Granulomatous inflammation in the aorta and its proximal branches—fibrous thickening of the aortic arch with obliteration of the mouths of the great vessels
• Other arteries can also be affected

Clinical features

• Slowly progressive course
• Diminished upper extremity pulses (pulseless disease)  ischemic symptoms in the arms
• Ocular and neurologic disturbances are common

Question 2

GIANT CELL ARTERITIS

Answer 2

General features
• In individuals above 50 years of age
• Pathomechanism: T-cell-mediated immune response to vessel wall antigen(s)

Morphology

• Segmental transmural granulomatous inflammation of head arteries, most often involving temporal arteries, but also ophtalmic or vertebral arteries
Consequence: stenosis or complete obliteration of the vessel

Clinical features

• Fever, fatigue, weight loss; sedimentation rate >50 mm/h • Palpable nodularity of the tortuous temporal artery
• Headaches, facial pain, jaw claudication during chewing • Visual symptoms + sudden unilateral visual loss  blindness
• Diagnosis: histological examination of biopsy specimen of the temporal artery