Page 44 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

20 David S. Younger

The risk factors for GCA-related ischemic events include visual loss, prior ischemic
events, marked intimal hyperplasia on temporal artery biopsy, elevated inflammatory
markers, older age at diagnosis, hypertension, ischemic heart disease, and absence of systemic
manifestations [16]. While there is no treatment to date that has been found to completely
reverse blindness in GCA once it has occurred, there is strong evidence to suggest that once
corticosteroids have been started the risk of visual loss is low [17]. For this reason,
corticosteroids should be started while the diagnostic evaluation is in progress and continued
for up to one year before tapering to the lowest maintenance levels.

Ohigashi and colleagues [18] ascertained an improved prognosis among 106 consecutive
patients with TAK in those with onset before 1999 compared to those diagnosed after 2000
(4.2% versus 0%) that was attributed to reduction in the time from onset to diagnosis,
replacement of digital subtraction angiography (79% versus 9%) with ultrasound (6% versus
34%), CTA (24% versus 77%), MRA (21% versus 57%), and 18F-FDG PET (0% versus
20%); less frequent complications of moderate or severe aortic regurgitation, and not
surprisingly, an increase in the use and maximal dose of corticosteroids (70% versus 97%);
and the use of first and second-line immunosuppressant agents (7% versus 42%). Surgical
treatment of TKA was similar between those with onset before 1999 and after 2000 (22.5%
versus 22.8%).

Medium Vessel Vasculitides

Polyarteritis Nodosa and Kawasaki Disease
Kernohan and Woltman [19] summarized the clinicopathological aspects of adult PAN at

postmortem examination, while Krahulik and colleagues [20] described fulminant childhood
PAN (cPAN), three decades after the first description of the first American patient by
Longcope in 1908 [21]. The dominant clinicopathological syndrome was peripheral neuritis
that occurred in one-half of patients early in the illness with a predilection for the legs. The
combination of acute and chronic lesions correlated with known exacerbations. Arteritic
lesions along nutrient arteries of the peripheral nerves were characterized by invasion of the
intima, media and adventitia by polymorphonuclear, plasma cells, eosinophils, and
lymphocytes associated with swelling of the media, fibrinoid necrosis, and fragmentation of
the internal elastic lamina (Figure 2). So impressed was Dr. Harry Lee Parker by the
frequency of arteritic lesions in the PNS, that he conceptualized nerve and muscle biopsy as a
useful mode for the diagnosis in life during a discussion of the paper by Kernohan and
Woltman [19]. Variants of cPAN were contemporaneously recognized in infants and young
children under the rubric of mucocutaneous lymph node syndrome, infantile PAN before
arrival at the preferred term KD [22-25] for the childhood syndrome affecting children of all
ages and races, with worldwide occurrence.

A retrospective study of 348 adult patients registered in the French Vasculitis Study
Group (FVSG) [26] who satisfied criteria for the diagnosis of PAN between 1963 and 2005
noted constitutional findings included fever, weight loss, myalgia, and arthralgia at
presentation in 93% of patients. PNS involvement included peripheral neuropathy and
mononeuritis multiplex in nearly equal proportion in 79%, and cutaneous involvement
notably, purpura, skin nodules, and livedo reticularis were noted in 50% of patients; CNS
involvement was noted in 5% of patients.

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