Page 314 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

288 Dimitri Chanouzas and Matthew David Morgan

Table 7. Chapel Hill Consensus Conference on the Nomenclature of Systemic
Vasculitis: Definitions

Granulomatosis with polyangiitis Granulomatous inflammation involving the respiratory
(Wegener) (GPA) tract, and necrotizing vasculitis affecting small to
medium-sized vessels (e.g. capillaries, venules,
Microscopic polyangiitis (MPA) arterioles, and arteries). Necrotizing glomerulonephritis
is common
Eosinophilic granulomatosis with Necrotizing vasculitis, with few or no immune
polyangiitis (Churg–Strauss deposits, affecting small vessels (i.e. capillaries,
syndrome) (EGPA) venules, or arterioles). Necrotizing arteritis involving
*Adapted from [2]. small and medium sized arteries may be present.
Necrotizing glomerulonephritis is very common.
Pulmonary capillaritis often occurs
Eosinophil-rich and granulomatous inflammation
involving the respiratory tract, necrotizing vasculitis
affecting small to medium-sized vessels, and
associated with asthma and eosinophilia

Clinical Features
The clinical features of AAV include constitutional symptoms such as malaise, joint

pains and weight loss, and other symptoms and signs depending on the extent of systemic
involvement. The upper and lower airways, skin, peripheral and central nervous system, as
well as the kidneys, are typically involved organ systems, accounting for approximately 5%
of patients with end-stage renal disease (ESRD) on renal replacement therapy [79].

Gastrointestinal Involvement
Abdominal pain and diarrhea in EGPA results from eosinophilic gastroenteritis and

mesenteric vasculitis leads to bowel ulceration, ischemia, and perforation [11]. Up to one-
third of patients with EGPA manifest GI involvement with severe involvement associated
with increased mortality [80]. The potential for GI involvement is varied and can include
upper or lower GI bleeding; perforation of the esophagus, stomach, small and large intestine;
bowel ischemia, colitis and cholecystitis, although the commonest manifestation is abdominal
pain [15]. Gastrointestinal involvement in GPA is uncommon, reported in between 0 and 24%
of patients [81-85]. Abdominal pain, diarrhea and blood loss are the commonest findings with
more severe GI involvement leading to ulceration and ischemia of the small and large
intestine while bowel perforation is a more frequent cause of acute abdomen directly
attributed to GPA [3, 81, 86-88].

Gastrointestinal involvement is uncommon in MPA. The GI manifestations of MPA can
include intestinal hemorrhage and ulceration, and intestinal ischemia as the presenting feature
of disease [89-91].

In 1996, the French Vasculitis Study Group (FVSG) developed a Five Factor Score (FFS)
shown in Table 8 that predicted the outcome in patients with PAN and EGPA. The presence
of one or more factors predicted a higher mortality than when absent [92]. The FFS was
revisited in 2011 in a retrospective analysis of 1108 consecutive patients with PAN, MPA,

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