Page 28 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

4 Eric L. Matteson

[1] as can those related to giant cell arteritis (GCA) by the oculist Ali ibn Isa in Baghdad in
1000 AD [2].

Vasculitis in Historical Context

The introduction of scientific methodology in the nineteenth replaced brought to a close
the romantic era of medicine and ushered in the scientific era, promoted by the advent of
university-based systematized anatomic pathology and technical developments notably
microscopic anatomy. Such advances were critical to the development of theories of the
cellular basis of disease embraced by Rudolf Virchow and others between 1848 and 1858 [3,
4]. Yet even in 1554, Antoine Saporta [5] provided the classical description of non-traumatic
macrovascular disease related to infectious disease due in the occurrence of syphilitic
aneurysms in an affected patient. Over time, inflamed vessels were frequently noted by
anatomic pathologists. By the early nineteenth century, infection was accepted as a cause of
vasculitis [6, 7] and other forms of vascular disease were more clearly delineated including
atherosclerotic disease and developmental arterial disease of the arteries [8].

The first clear description of non-infectious vascular inflammation was likely by Joseph
Hodgson in 1815 [9] in his inspection of large arteries at postmortem examination and the
recognition of arterial intimal inflammation suggesting that such changes could result from
high intravascular pressure, trauma, and a systemic inflammatory state. Moreover, vascular
inflammation previously related only to syphilitic infection, could also be associated with
other diseases [9]. During the same era, inflammation of the veins was described by John
Hunter [10].

The advent of microscopy enabled further investigation of the nature of vascular
inflammation and its origins. The anatomic pathologist Karl von Rokitansky, who viewed
arteritis as having its origin in the adventitia, was unlike Virchow who employed careful
microscopic studies of his anatomic specimens and postulated that inflammation instead
began in the intima and media [3, 11].

Polyarteritis Nodosa

The first clearly recognizable patient with idiopathic vasculitis of the polyarteritis nodosa
type was described by Adolf Kussmaul and Rudolf Maier in 1865 [12] in a 27-year-old
journeyman, the findings of whom influenced and systematized later observations. The
patient, Carl Seufarth had improved from a prior infectious illness one month earlier when he
was arrested in destitute circumstances after wandering into the city of Freiburg, in the Black
Forest of Germany during the summer of 1865, and taken to the municipal jail where the
officers recognized his poor medical state. He was later transferred to the University of
Freiburg Internal Medicine Clinic where he was and was able to walk up the two flights of
stairs to Dr. Kussmaul?s office, where he examined him, and thereafter hospitalized.

Over the ensuing months, Seufarth developed fever, generalized muscle aching,
mononeuritis multiplex of the arms and legs, abdominal pain, and proteinuria.

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