While diseases of the blood vessels have been recognized since antiquity, inflammation of the vessels has only been recognized as a distinct clinical entity for about the past 150 years. Polyarteritis nodosa was the first noninfectious vasculitis to be described and studied in detail. In large measure, most forms of vasculitis described subsequently have been characterized and classified on the basis of features either similar to or distinct from polyarteritis.
Knowledge of the original case descriptions of polyarteritis nodosa and their historic context provides a foundation for understanding current concepts of this disease. They are a valuable reference point for the current efforts at diagnosing, treating, and classifying vasculitis, and emphasize both the importance of careful clinical observation to these efforts and the essential nature of medical science.
Kussmaul and Maier gave the first complete description of this form of systemic necrotizing vasculitis in 1866 and assigned the name "periarteritis nodosa" to it (1). They distinguished this "unique arterial disease" from infectious disorders, including trichinosis, diphtheria, and especially syphilis. Syphilitic aneurysm of the aorta had first been described by the Montpellier professor Antoine Saporta in 1554 (2). Joseph Hodgson more clearly related syphilis to aortitis in his Treatise on Diseases of Arteries and Veins published in London in 1815
(3). This book likely gave the first clearly defined example of arteritis.
Prior to the report of Kussmaul and Maier, several other authors had described aneurysmal lesions of the arteries that may have been inflammatory but not syphilitic in nature, but in most cases the clinical and pathologic-anatomic information is not sufficient to conclude that the condition represents polyarteritis nodosa. In the 18th century, Joannes Gottlieb Michaelis reported on a man who had had dilated arteries on the left arm beginning in childhood (4), and Antonio Matani reported on a patient with innumerable aneurysms throughout the entire body (5). Philippe-Joseph Pelletan described a patient found to have 63 arterial aneurysms; the patient died of a wasting disease (6).
Early perspectives on vascular inflammation
Diseases of the blood vessels, especially those associated with inflammation, remained relatively obscure until John Hunter in London directed attention to them through his observation on phlebitis at the end of the 18th century (7). Indeed, Hunter was the first to recognize inflammation of the veins, and also the first to demonstrate the muscularity of arteries. He postulated that arterial aneurysms were due not simply to weakness of the arterial wall but to actual disease of the artery (8).
After Hunter's work, interest in inflammation of the blood vessels grew rapidly, and soon persistent fevers were often attributed to inflammation of the arteries. This view seemed to be supported by the report of Johann Peter Frank, working in Pavia and Vienna, on the gross macroscopic appearance of red and inflamed inner walls of the large arteries, heart,