Page 313 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Systemic Vasculitis of the Gastrointestinal Tract 287

It is usually self-limiting and characterized by the tetrad of palpable purpura in the absence of
thrombocytopenia or coagulopathy; arthritis or arthralgia; abdominal pain; and renal
involvement. The disease follows a more severe course when it presents in adults with a
higher incidence of renal impairment and end-stage renal disease [68].

Diagnosis
Although childhood IgAV/HSP is typically clinically diagnosed, adults generally undergo

skin or renal biopsy for tissue confirmation. Imaging can be helpful in assessing the
gastrointestinal manifestations of IgAV/HSP especially ultrasonography for the detection of
increased bowel wall thickness, hematomas, and intussusception [69].

Gastrointestinal Involvement
Gastrointestinal occurs in 50% of children with IgAV/HSP including, intussusception,

intestinal ischemia and infarction, intestinal perforation, gastrointestinal hemorrhage, late ileal
stricture, fistulae formation, acute appendicitis, pancreatitis, and hydrops of the gallbladder
[15]. Gastrointestinal symptoms occur within eight days of the appearance of the rash
although they may precede the rash. A 2001 Spanish study [70] noted that 16.7% of affected
children presented with abdominal pain as the solitary symptom; 7.6% presented with
abdominal pain and a rash, and 2.6% with abdominal pain, rash and joint pains. Altogether,
70.5% of patients progressed to mesenteric ischemia, and 30.8% had evidence of GI bleeding.
The commonest GI manifestation in childhood IgAV/HSP was intussusception. Although the
prognosis of HSP in children is good, adult onset HSP often has a less favorable outcome
[68]. In adults severe gastrointestinal involvement, manifested by abdominal pain and GI
bleeding [71-73] can be associated with a fatal outcome [74-76].

Treatment
Management of IgAV/HSP is largely supportive with administration of non-steroidal

anti-inflammatory drugs (NSAID) and corticosteroids for severe abdominal pain and joint
involvement. There is no convincing evidence to suggest that early use of corticosteroids
reduces the risk of renal involvement or acute gastrointestinal complications [77]. The
management of intussusception in children with HSP often involves surgical correction [78].

ANCA-Associated Vasculitis

Epidemiology and Classification
The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a

group of autoimmune inflammatory conditions that predominantly affect small- to medium-
sized blood vessels, comprising GPA, microscopic polyangiitis (MPA), and eosinophilic
granulomatosis with polyangiitis (EGPA).

The individual annual incidence of the AAV is less than 10 per million persons with a
peak age at onset in the seventh to eighth decades [79]. Table 7 lists classification criteria for
the AAV [12].

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