Page 311 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Systemic Vasculitis of the Gastrointestinal Tract 285

Table 6. Clinical Features of Polyarteritis Nodosa*

Manifestation Specific Problems Frequency (%)
Systemic symptoms Fever, malaise, weight loss 80
Neuropathy Mononeuritis multiplex, polyneuropathy 75
Arthralgia and / or myalgia Articular and / or diffuse extremity pain 60
Cutaneous Livedo reticularis, purpura, ulcers 50
Renal disease Elevated creatinine, hematuria, 50
glomerulonephritis
Gastrointestinal symptoms Abdominal pain, rectal bleeding 40
Hypertension New onset 35
Respiratory manifestations Infiltrates, nodules, cavities 25
Central nervous system Stroke, confusion 20
disease
Orchitis Testicular pain, swelling 20
Cardiac involvement Cardiomyopathy, pericarditis 10
Peripheral vascular disease Claudication, ischemia, necrosis 10
*Adapted from [46].

Gastrointestinal Involvement
Gastrointestinal involvement occurs in 40% to 65% of patients with PAN. Abdominal

pain is the commonest reported GI symptom in PAN. Gastrointestinal involvement can arise
from stenotic lesions that lead to bowel wall ischemia and aneurysm formation that results in
hemorrhage. Necrosis of the bowel wall with the potential for subsequent perforation can
develop if systemic inflammation gives rise to transmural ischemia and confers a poor
prognosis [51]. Gastrointestinal manifestations of PAN include ulceration, obstruction,
infarction, perforation and hemorrhage that can affect the stomach, small bowel, large bowel
and the appendix [53-55]. A retrospective analysis of 54 patients with PAN found GI
involvement in 24 (44%) with two-thirds noting abdominal pain at presentation, while all
others had abdominal pain at some point in their illness. About one-half of patients developed
acute abdomen with associated diagnoses of bowel infarction and perforation and less
commonly acute cholecystitis, gall bladder infarction, aneurysmal ruptures of hepatic, splenic
or renal arteries, and bleeding from perforated gastric ulcers [56]. Multiple aneurysm
formation is a defining feature of PAN so noted in 50% to 70% of those with involvement of
the GI tract with the hepatic artery affected in 50% to 60%; splenic artery in 45%; and
pancreatic artery in 25 to 35% [56-58]. Massive internal hemorrhage due to rupture of an
aneurysm within the intestine is rare but nonetheless carries a high mortality (75%) [58-60].

Treatment
The mainstay of treatment for mild PAN is corticosteroids however gastrointestinal

involvement usually necessitates treatment with cyclophosphamide, as it constitutes severe
disease and has been shown to have a better outcome when treated with a combination of
cyclophosphamide and corticosteroids [61]. PAN secondary to HBV infection is treated with
two weeks of corticosteroids followed by antiviral treatment combined with plasma exchange.
Those so treated with antiviral medication do not experience HBV relapses once viral
replication has stopped and seroconversion has been achieved [50]. Occlusive mesenteric

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