Page 29 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

History and Background of Vasculitis 5

He died on May 30, 1865 and an English account of his case was later published [13].

Working closely together with Kussmaul, the anatomic pathologist Rudolf Maier, at the
University of Freiburg performed detailed postmortem microscopic examination and

described the findings under the rubric of periarteritis nodosa, and later polyarteritis nodosa.
In their words, there was a “peculiar, mostly nodular thickening (periarteritis nodosa) of
countless arteries of and below the caliber of the liver artery and the major branches of the

coronary arteries of the heart, principally in the bowel, stomach, kidneys, spleen, heart, and

voluntary muscles and, to a lesser extent, also in the liver, subcutaneous cell tissue, and in the
bronchial and phrenic arteries” [12]. The nodular thickened vessels revealed inflammatory
changes in the media and adventitia. The kidneys had changes of “acute Bright?s disease.”
Maier wrote, “The change affects the intralobular arteries, which have glomeruli at their
bifurcations, and extends into these branches and even into the glomeruli” [12].

Kussmaul and Maier ascribed the pathological alterations in the arteries to “inflammation

of the arteries affecting principally the perivascular sheath, in which the media also had a
part, at least in its outer layers,” recognizing that the inflammatory changes “often attacked

neighboring tissues in the opposite direction, for example, the renal parenchyma, connective
and muscle tissues.” The investigators initially considered the disease to be a result of
infectious causes including a worm infestation, both because of the peculiar nature of the pea-
size nodules which appeared in the tissue below the epidermis of Seufarth?s abdomen, and the

thickened fibrotic nature of the affected vessels. Indeed, an abstract cited their patient as an
example of nematode aneurysmal disease before Maier [14] conducted a later thorough

microscopic evaluation.

There may even have been earlier cases of polyarteritis nodosa (PAN), but none were so

recognized in the medical literature. Karl von Rokitansky provided a very brief clinical
description of a patient seen in 1852 at the University of Vienna found to have fatal

aneurysmal coronary and mesenteric arterial changes [15] believed to be an early example of

PAN.
However Rokitansky did not undertake microscopic examination of the tissue, and it was

not until his student Hans Eppinger later examined the specimens microscopically that it was

clear that the patient had polyarteritis nodosa [16]. Kussmaul did not consult Rokitansky
about his earlier patient because of his unfavorable experience as a visiting clinician in
Rokitansky?s Institute of Anatomic Pathology institute at about 1840. Instead he collaborated

with Virchow who affirmed that while he might have seen a similar patient but did
understand its fundamental nature [12, 17]. Attesting to the rarity of the disorder, only about

30 additional cases were described in Europe and the United States in the ensuing four

decades [17-19], and into the twentieth century.

Necrotizing Vasculitis

The first cases of vasculitis were macroscopically apparent and could be assessed with
the naked eye. A clear understanding of microscopic necrotizing vasculitis did not emerge
until well into the twentieth century. Davson in 1948, and Wainwright in 1950, provided the
first English language accounts of microscopic polyarteritis or polyangiitis (MPA) that
affected small arterial capillaries and venules, particularly in the kidney and lungs, often
associated with necrotizing glomerulonephritis [20, 21].

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