Page 205 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Classification and Pathogenicity of ANCA-Associated Vasculitis 181

Central and Peripheral Nervous System

AAV can affect the peripheral (PNS) and central nervous system (CNS) so noted in 19%
of patients with GPA and 17% of those with MPA at presentation [7]. Involvement of the
PNS usually presents with motor or sensory deficits in a mononeuritis multiplex or distal
sensory polyneuropathy distribution.

While typically painless in the acute stage, pain syndromes commonly develop as a result
of neurodegeneration. Involvement of the CNS occurs in 8% of patients with AAV at
presentation [13] manifesting as weakness, dysphagia, blindness or other CNS disturbances
[25]. Three distinct patterns of CNS involvement have been identified; during the generalised
phase the small and medium sized vessels of the CNS can become inflamed; secondly,
localised granulomatous masses from the upper respiratory tract may invade the CNS; thirdly,
granulomata may develop within CNS structures [26].

Cutaneous

Cutaneous involvement occurs in 42% of GPA and 25% of MPA patients at presentation
[7]. A wide range of signs are seen that include purpura, papules, urticaria, infiltrative
erythema, infarction and splinter hemorrhage, ulcerated nodules, livedo reticularis, and digital
gangrene [27]. The type of lesion is determined by the size of the blood vessel affected. The
most common manifestation is a palpable purpuric rash beginning along the lower extremities
that may become ulcerated or vesicular. The urticarial rash seen in vasculitis can be
differentiated from allergic nonvasculitic urticaria by the fact that it will usually last for more
than one day and may progress to purpuric lesions [28].

Cardiac

Direct cardiac involvement is uncommon in either GPA or MPA; however the former
may be associated with coronary arteritis, pericarditis and valvular disease. Endocarditis can
occur as a result of aortic valvular lesions [29]. MPA may be associated with pericarditis,
heart failure and hypertension however the incidence is very low [30].

Gastrointestinal

Gastrointestinal involvement occurs at presentation in up to 30% of patients with AAV
[12, 31-33]. Gastrointestinal involvement may present with ischemic or vasculitic colitis,
bloody diarrhea, pain and progression to ulceration, perforation and infarction [9, 34]; 30 to
56% of patients with MPA can have GI involvement [35, 36] the commonest presenting
symptom of which was pain, so noted in 97% of patients, followed by nausea, vomiting,
diarrhea, melena, and hematemesis [37].

Rarely, other intra-abdominal organs can be involved such as the gall bladder and
pancreas [34].

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