Page 316 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

290 Dimitri Chanouzas and Matthew David Morgan

Other Secondary Vasculitides

Gastrointestinal complications occur in other autoimmune conditions such as rheumatoid
arthritis (RA) and mixed connective tissue disease (MCTD) due to secondary vasculitis. In
patients with RA vasculitis, up to 38% of cases have involvement of the mesenteric vessels
leading rarely to ischaemic ulceration and bowel perforation [97]. Secondary vasculitis
leading to gastrointestinal involvement in MCTD is rare and occurs in the form of small and
large bowel ulceration and perforation [98, 99] as well as fatal gastrointestinal hemorrhage
[100]. Vasculitic gastrointestinal manifestations present in patients with Hepatitis C virus
(HCV) infection, as well as non-infectious mixed cryoglobulinaemic vasculitis (CV) ranging
from abdominal pain to intestinal bleeding, infarction and perforation, wherein the latter GI
involvement is associated with increased mortality [101]. Gastrointestinal involvement was
described in vasculitis secondary to the drugs. Propylthiouracil [102] and hydroxyurea [103],
or infection associated with cytomegalovirus (CMV) [104].

Treatment-Related GI Side Effects

As the treatment of vasculitis often involves immunosuppression with drugs such as
corticosteroids, cyclophosphamide, azathioprine, and methotrexate and in recent years
biological therapies such as rituximab, treatment-related involvement of the GI tract must be
considered. Reactivation of CMV can lead to significant gastrointestinal problems in the
context of immunosuppression. Symptoms of abdominal pain, diarrhea and bleeding, as well
as endoscopic signs of erythema, nodules and ulceration, are non-specific but similar to those
of vasculitis [11]. It is very important to secure the diagnosis with biopsy specimens and
peripheral blood CMV viral load as the latter usually responds to anti-viral therapy. Candida
involvement of the oral cavity and the esophagus is a recognized complication of high-dose
corticosteroid treatment. Therefore prophylaxis with a topical anti-fungal preparation is
advocated for patients on such treatment regime [105]. Finally, corticosteroids and NSAID
can lead to gastroduodenal ulceration as well as small and large intestine enteropathy [11].

Conclusion

Gastrointestinal involvement in vasculitis largely reflects the size of the blood vessel
involved. Large vessel involvement may lead to stenotic lesions manifesting as ischemia or
infarction of large, but often unusual segments of the GI tract. Medium vessel involvement is
typified by aneurysmal as well as stenotic disease that can produce ischemia or infarction and
bleeding whilst small vessel disease can present with abdominal pain, patchy ulceration,
infarction and bleeding as well as intestinal perforation.

Treatment is aimed at controlling the underlying vasculitic process with
immunosuppression although open surgical or endovascular therapy is important in the
management of mesenteric vasculitis. Severe gastrointestinal involvement in vasculitis is
associated with a higher mortality.

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