Page 31 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

History and Background of Vasculitis 7

Large Vessel Vasculitis

Patients with large vessel vasculitis or pulseless disease have been described for the past
3000 years [8]. Early descriptions of the condition were initially related to trauma while those
in the late eighteenth and nineteenth centuries were causally associated with arterial sclerosis
[4, 33, 34]. The formidable anatomic pathologist Morgnani [35] described an approximately
40-year-old woman with absent radial pulses for at least six years prior to her death in which
postmortem examination showed ectasia of the proximal aorta with stenosis of the lower
portion, and histologically normal radial artery vessels.

In 1839, Davy [36] described two patients with likely large vessel vasculitis including a
55-year-old male who presented with weak arm pulses and later vertigo. The physical
findings were of “a throbbing pulse at the upper part of the sternum, and a slight prominence
of the bone there to some little extent”. Davy [36] suspected an aortic arch aneurysm, which
was indeed found one and one-half years later at postmortem examination. So stated, a large
aneurysm of the aortic arch was found and “all the great vessels arising from the arch were
completely closed up at their origin”. Davy [36] described absent arm pulses in a 36-year-old
man who like the first patient, was a soldier in the British Army. Postmortem examination in
the second patient showed aortic arch dilatation and occlusion of the left subclavian and
carotid arteries.

A further description from this era was given by Savory [37] in 1854 of a 22-year-old
woman who was ill for five years with pulseless arteries of the arms and neck, and blindness
over the ensuing year. Postmortem examination in that patient showed marked stenosis of the
aortic arch and it branches, without aneurysms. Savory [37] was probably aware of the
previous descriptions by Rokitansky [15], and further differentiated his patient by ascribing
the observed inflammation to an origin in the intima and media, rather than the adventitia.

It has been speculated that Ali ibn Isa [2] may have described a patient with temporal
arteritis at about 1000 AD when reporting, “one treats not only migraine and headache in
those patients that are subject to chronic eye disease but also acute, sharp, catarrhal affections,
including those showing heat in and inflammation of the temporal muscles.

These disease conditions may terminate in loss of eyesight; frequently, they are attended
by a considerable degree of chemosis.” While it remains unclear whether this actually
represents a case of GCA, there is no doubt that in 1890, Hutchinson [38] provided the first
clinical description of what is truly regarded as contemporary temporal arteritis when he
reported a “peculiar form of Thrombotic Arteritis of the aged, which is sometimes productive
of Gangrene”, in an 80-years-old man. His patient, a servant named Rumbold, presented with
headache and “red streaks on his head, which were painful and prevented him from wearing
his hat”. Hutchinson [38] recounted that that the patient lived for several years without “any
other manifestation of arterial disease.”

The clinical syndrome and histological aspects of GCA was clearly delineated by Horton,
Magath, and Brown [39] at the Mayo Clinic in 1932. Indeed, they obtained the first biopsies
of the affected temporal arteries in living patients and described the typical findings including
“peculiar circumscribed areas of what appear to be granulation tissue…in the adventitia of the
blood vessels, which suggested granulomas,” stating further that “this represented the most
characteristic lesion present”. Like Hutchinson [38], of whose work they apparently were not
aware, these investigators [39] initially regarded the disease as benign, as “complete recovery
occurred in each case”, however on further follow-up, they observed that two of their first

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