Page 32 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

8 Eric L. Matteson

patients died within two years of what they termed “unrelated conditions”. By 1938, visual
loss was associated with giant cell arteritis by Jennings [40]. The association of headache
with occasional jaw claudication prompted the term “cranial arteritis” in 1946 by Kilbourne
and Wolffe [41].

Much earlier in 1908, Takayasu [42] described a young woman with peculiar retinal
artery changes and “wreath-like anastomosis surrounding the optic disc at a distance of 2 or 3
mm, and surrounding this was another circular anastomosis”. Takayasu [42] described
“lumps” in the surrounding vessels that were seen to “move from day-to-day.” These findings
were discussed at the 12th Annual Meeting of the Japanese Society of Ophthalmology
whereupon other discussants noted the relation of pulseless radial arteries to the retinal artery
changes in their own patients, an association that Takayasu had not appreciated [42].

By 1925, Beneke [43] reported the first comprehensive histopathologic analysis of
affected large vessels in patients with pulseless disease of the Takayasu arteritis type. There
was virtually complete medial necrosis, intimal sclerosis, adventitial scarring and thickening
of large arteries at postmortem examination in affected patients. More importantly, Beneke
[43] described giant cells which he related instead atheromatous changes and white blood cell
infiltrates of greater importance to the disease pathogenesis.

Other Forms of Idiopathic Vasculitis

Henoch-Schönlein Purpura
The disease known as Henoch-Schönlein purpura likely first appeared in a report by

William Heberden [44] in 1801 in the account of a 4-year-old with purpuric lesions of the leg,
buttock, and scrotum; as well as in a 5-year-old with similar lesions and abdominal pain.
Ollivier [45] described a youngster with purpura and abdominal pain in 1827, but the distinct
disease awaited the description of “peliosis rheumatica” by Schönlein in 1837. In 1874,
Henoch [46] reported four affected children with joint pain, purpuric rash, abdominal pain,
and diarrhea recognizing the potentially fatal aspect of the disease. The cause of the disease
has been ascribed to infection, although a specific infectious agent has yet to be definitively
identified. In 1915, Frank [47] postulated an allergic cause so termed “anaphylactoid
purpura”. Glanzmann [48] postulated that the combination of infection and hypersensitivity
culminated in the observed findings of purpura, nephritis and abdominal pain, although a
potentially causative antigen was not identified.

Behçet Disease
Oral and genital mucosal ulcers and eye inflammation of Behçet disease may have been

recognized by Hippocrates but was certainly described by Blüthe [49] in 1908, Planner and
Remenovsky [50] in 1922, Shigeta [51] in 1924, and Whitwell in 1934 [52]. One of latter
described patients [52], a 29-year-old woman, with oral and genital ulcers, likely erythema
nodosa, and venous emboli of the leg, had the cardinal features of so called Adamantiades-
Behçet. Adamantiades [53] described a 22-year-old soldier patient with oral and genital
ulcerations who experienced recurrent iritis and hypopyon, and was thought to have syphilis
on the basis of a positive Wassermann reaction. Treatment for syphilis was unsuccessful.

In his initial description, Behçet [54] described two patients, a 34-year-old woman and
40-year-old man with recurrent oral and genital aphthous ulcerations, uveitis and hypopion.

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