Page 237 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

212 Loic Guillevin

Ophthalmologic Manifestations

Ocular manifestations of necrotizing vasculitis were noted in 42/393 (10.7%) of patients
with PAN [71] the commonest features of which included blurred vision in 31%, sudden
visual loss in 19%, retinal vasculitis in 16.7%, and uveitis in 11.9% of patients. Scleritis,
episcleritis and other manifestations are rarely observed.

HBV-Related Manifestations

An onset before age 40 years, concomitant malignant hypertension, renal infarction,
orchi-epididymitis, gastrointestinal manifestations, and surgical emergencies were all more
frequent in HBV-associated infection. Sergent and coworkers [72] noted that two of the three
deaths among nine patients were attributed to colon vasculitis. Ischemia was responsible for
gastrointestinal and renal manifestations that were due to microaneurysm formation and
infarction noted on visceral angiography. Effectively treated, microaneurysms regress on
serial angiography [73] that may result from microaneurysmal thrombosis with evolution to
fibrosis. Hepatic cytolysis is usually moderate and cholestasis is minor or absent.
Liver biopsy pathology exhibits signs of chronic hepatitis even when PAN becomes manifest
a few months after HBV infection. HBV-related PAN is acute and initially severe but the
outcome is excellent for most patients when adequate treatment is associated with
seroconversion. The sequela of vascular nephropathy is similarly favorable in those patients
able to obtain substantial recovery with little residual functional renal impairment.

Childhood PAN

PAN is the commonest systemic necrotizing vasculitis in children after KD and Henoch–
Schönlein purpura (HSP) [74]. Neither the ACR criteria [4] nor the 2012 Revised CHCC
Nomenclature [5] were validated for children. The European League against
Rheumatism/Pediatric Rheumatology European Society (EULAR/PReS) proposed specific
classification criteria for juvenile vasculitides that are under review by the ACR (Table 4)
[75].

The EULAR/PReS criteria [75] were based mainly on a literature review and a
consensus-based process. The primary objective of the 2008 Ankara Consensus Conference
was to validate the EULAR criteria for pediatric vasculitides. The final classification criteria
of classic childhood PAN (cPAN) required systemic inflammatory disease with mandatory
evidence of necrotizing vasculitis or angiographic abnormalities of medium-, to small-sized
arteries plus one of the following five criteria including, skin involvement, myalgia or muscle
tenderness, hypertension, peripheral neuropathy and renal involvement [76]. The final
EULAR/PReS childhood PAN classification criteria achieved a sensitivity of 89.6% and a
specificity of 99.6%.

The mean age at diagnosis of cPAN is age 9 ± 4 years, with an equal ratio of boys and
girls. Clinical manifestations fall within the same spectrum as for adults, except for more
frequent cutaneous involvement in children. Indeed, 80% of affected children manifest skin
involvement, with a limited cutaneous form accounting for one-third of cases. Prognosis is

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