Page 33 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

History and Background of Vasculitis 9

Behçet [54] speculated an infectious viral cause, undertaking a detailed examination for the
presence of viral products whereas a vasculitic etiopathogenesis was not contemplated.

Kawasaki Disease

In 1961, childhood febrile mucocutaneous lymph node syndrome was first described by

Kawasaki [55] in Tokyo. The suspected causes included allergic, infectious, and autoimmune

mechanisms. In a subsequent report in 1974 based on more than 6000 patients seen in Japan,
Kawasaki [56] indicated he noted “infantile periarteritis nodosa-like arteritis of the coronary
artery accompanied by thrombosis and aneurysm…” Kawasaki disease is increasingly

regarded as an infantile/juvenile form of polyarteritis nodosa.

Single Organ Vasculitis
A multitude of single organ vasculitides have been recognized in the past several

decades. A cutaneous form of polyarteritis was described by Diaz-Perez and Winkelmann
[57] at Mayo Clinic in 1974. A non-systemic vasculitic neuropathy was described by Dyck
and coworkers at the Mayo Clinic in 1986 [58]. Granulomatous angiitis (GANS), isolated
angiitis of the central nervous system (IACNS) and primary angiitis of the CNS (PACNS) are
equivalent terms for a potentially lethal adult CNS vasculitis, clinically and pathologically
characterized beginning with the description by Cravatio and Fegin in 1959 [20, 59]. A
similar disease affects children although curiously without granulomatous pathology. The
occurrence of idiopathic vasculitis in isolated organs which has become increasingly
recognized, and on occasional may evolve to affect other organs, was included in the 2012
Revised Chapel Hill Consensus Conference (CHCC) Nomenclature of Vasculitides [60].

Since the initial description by Kussmaul and Maier [14], secondary forms of vasculitis
due to hypersensitivity, infection, and vasculitis occurring in the context of other autoimmune
rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus have also
been described. Vasculitis has also been related to the presence of systemic cancer. Indeed in
1958, Guichard [61] introduced the term “Paraneoplastic" to describe the latter occurrence.

Classification of Vasculitis

Early attempts to categorize the vascular diseases were based upon the likeliest
etiological postulates to explain a given gross anatomical feature, as illustrated in
Rokitansky?s [11] nineteenth century classification of aneurysms that included those arising
as a result of increased blood pressure and aging, non-atheromatous muscularis inflammation,
trauma, pseudomembranous intimal proliferation of the intima or adventitial weakness of the
intima at the site of atheroma. In 1952, Zeek [62, 63] offered the first modern classification
system of vasculitis, dividing them simplistically into hypersensitivity and angiitis, allergic
granulomatous angiitis, rheumatic arteritis, periarteritis nodosa, and temporal arteritis;
granulomatosis and polyangiitis and Takayasu arteritis were not included. In 1990, the
classification of vasculitis by the American College of Rheumatology (ACR) distinguished
one form another on the basis of review of nearly1200 cases of vasculitis [64].

The 1994 [60] and 2012 Revised CHCC [65] provided better systematization and
nosology, recognizing both primary idiopathic vasculitis and secondary vasculitis including

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