Page 315 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Systemic Vasculitis of the Gastrointestinal Tract 289

EGPA and GPA registered in the FVSG database; age > 65 years, cardiac symptoms,
gastrointestinal involvement and renal insufficiency, which were all independently associated
with a higher five year mortality [93].

Table 8. The Five Factor Score*

Factor Definition
1 Renal involvement Proteinuria > 1g / 24 hours
2 Renal impairment Serum creatinine > 1.582 mg/dl
3 Gastrointestinal involvement Presence of any of the following:
bleeding, perforation, infarction
4 Central nervous system involvement pancreatitis
5 Cardiac involvement
*Adapted from [86]. Presence of cardiomyopathy

Treatment
Management of AAV entails induction of disease remission followed by maintenance

treatment and therapy directed at disease relapses. Different immunosuppressive agents can
be used depending on disease severity ranging from methotrexate for localized and early
systemic disease, to a combination of high dose corticosteroids, plasma exchange and
cyclophosphamide or rituximab in severe life-threatening disease [79].

Secondary Vasculitides

Systemic Lupus Erythematosus

SLE is a systemic autoimmune condition that can affect any part of the body and GI tract.
Vasculitis secondary in SLE, which can affect any part of the GI tract appears to have a
predilection for the superior mesenteric artery [3]. Incipient ischemic and infarction can result
in secondary ulceration, bleeding, stricture formation, and perforation [94].

Variable Vessel Vasculitis

Behçet Disease

Behcet disease (BD) is a non-specific necrotizing vasculitis that affects multiple organs
and is characterized by orogenital ulceration, arthritis, uveitis and frequent peripheral nerve
and GI involvement. Gastrointestinal involvement occurs in approximately 10 to 40% of
patients [3]. Frequently involved sites include the ileocecal area and the esophagus [11].
The hallmark of BD is ulceration that is often deep and can penetrate through the bowel wall
leading to perforation, hemorrhage and fistula formation [95, 96].

Complimentary Contributor Copy