Rheumatology Journal Club

Gut vasculitis
By Dr Nur Hidayati Mohd Sharif

Rheumatology Dept Hospital Selayang 11/3/2011

Pathophysiology
 Sx of systemic vasculitis involving the
gastrointestinal tract result from mesenteric
ischemia from small and medium sized
vessels
 Medium-vessel vasculitis

◦ polyarteritis nodosa and Kawasaki syndrome.

 Small-vessel vasculitis
◦ microscopic polyangiitis, Wegener granulomatosis,
Churg-Strauss syndrome, Henoch-Schönlein
syndrome, systemic lupus erythematosus,
rheumatoid vasculitis, and Behçet disease.
62 patients with systemic small and medium-
sized vessel vasculitides and gastrointestinal
tract involvement followed between 1981 and
2002.
 46 men and 16 women (male:female ratio,

2.9)
 38 polyarteritis nodosa (21 related to

hepatitis B virus)
 11 Churg-Strauss syndrome
 6 Wegener granulomatosis
 4 microscopic polyangiitis
 3 rheumatoid arthritis-associated vasculitis.

Pagnoux, C et al Medicine
(Baltimore) 2005; 84:115.
 GI manifestations were present at or occurred
within 3 months of diagnosis in 50 (81%)
patients
- abdominal pain - 61 (97%)
- nausea or vomiting in 21 (34%)
- diarrhea in 17 (27%)
- hematochezia or melena in 10 (16%)
- hematemesis in 4 (6%)

Pagnoux, C et al Medicine
(Baltimore) 2005; 84:115.
 Gastroduodenal ulcerations 17 (27 %) patients
esophageal in 7 (11%), and colorectal in 6 (10%),
but histological signs of vasculitis were found in
only 3 colon biopsies.
 21 (34%) patients had a surgical abdomen

11 (18%) developed peritonitis

9 (15%) had bowel perforations
10 (16%) bowel ischemia/infarction, 4 (6%)
intestinal occlusion, 6 (10%) acute appendicitis, 5
(8%) cholecystitis, and 3 (5%) acute pancreatitis.
 Sixteen (26%) patients died.

Pagnoux, C et al Medicine
(Baltimore) 2005; 84:115.
 For 21 pt with surgical cx
10-month survival rate - 71% (95% confidence interval
[CI], 52-90)
5-year survival rates - 56% (95% CI, 35-77)
 For 41 patients without surgical abdomen
10-month survival rate - 94% (95% CI, 87-101)
5-year survival rates - 82% (95% CI, 70-94)
(p = 0.08).
 Gastrointestinal manifestations significantly associated
with increased mortality in multivariate analysis
Peritonitis (hazard ratio [HR]= 4.3, p<0.01)
Bowel perforations (HR = 5.7, p<0.01)
Gastrointestinal ischemia or infarctions (HR = 4.1,
p<0.01)
Intestinal occlusion (HR = 5.5, p<0.01)
Imaging
 Abdominal x-ray & U/S – can be normal
 CT & MRI especially with angiography

◦ bowel wall thickening with or without the target sign

 alternating layers of high and low attenuation within the
thickened bowel wall due to submucosal edema or hemorrhage
◦ intramural pneumatosis, mesenteric or portal venous gas
◦ mesenteric arterial or venous thromboembolism
◦ bowel wall become thinned or occasionally invisible when
the involved bowel segment becomes gangrenous
◦ engorgement of mesenteric veins and mesenteric edema
◦ lack of bowel wall enhancement
◦ increased enhancement of the thickened bowel wall
◦ bowel obstruction
◦ infarction of other abdominal organs
Endoscopy
 “vasculitis-like” hemorrhage, small ulcers
and edema of the duodenum
 Other GI or extra-intestinal vasculitis or
angiographic abnormalities (seen in 67% of
the 39 patients who underwent angiography)
not predictive of surgical complication or poor
outcome
 Prognosis has dramatically improved during

the past 30 years probably due to :-

- better management of these more severely
ill patients
- prompt surgical intervention when indicated
- the combined use of steroids and
immunosuppressants
PAN
 Affects small and medium sized arteries
 7 % associated with hepatitis B but only 1

percent of patients with HBV develop

polyarteritis
◦ develops within 12 months of the initial infection
◦ result of deposition of viral antigen-antibody
complexes in vessel walls
◦ 1-year survival for patients with HBV-associated
PAN is lower  increased incidence of
gastrointestinal bleeding and perforation
 Gastrointestinal involvement
◦ occurs in 14-65 percent of patients with PAN
◦ gallbladder and small bowel  most frequent sites
 The more severe manifestations include :-
- intestinal infarction or perforation
- pneumatosis intestinalis
- pseudomembranous colitis
- cholecystitis
 Series of 16 patients hospitalized with severe PAN
◦ 5 developed an abdominal crisis related to the disease
◦ At laparotomy, gross evidence of mesenteric arteritis with
infarcted bowel and intestinal perforation.
◦ All five patients died despite surgery and medical therapy.
 In another series of 165 patients with PAN or
Churg-Strauss syndrome
◦ 31 percent of deaths during follow-up were attributable to
gastrointestinal disease (gastrointestinal bleeding or
peritonitis in 11 and pancreatitis in 2)
 Rx with corticosteroids and cyclophosphamide
- improves survival and symptoms relief
 For patients with PAN related to HBV infection
or hairy cell leukemia – treatment of
concurrent disease is indicated
 Some patients with PAN have chronic GI sx
during inactive period
◦ Chronic inflammation of vessels led to progressive
narrowing of the vascular lumens and true
arteriosclerosis.
 Figure 3a.  Polyarteritis nodosa in a 39-year-old man with a 3-month history of fever, myalgia,
arthralgia, and peripheral neuropathy.

Ha H K et al. Radiographics 2000;20:779-794

©2000 by Radiological Society of North America

 Figure 3b.  Polyarteritis nodosa in a 39-year-old man with a 3-month history of fever, myalgia,
arthralgia, and peripheral neuropathy.

Ha H K et al. Radiographics 2000;20:779-794

©2000 by Radiological Society of North America

 Figure 3c.  Polyarteritis nodosa in a 39-year-old man with a 3-month history of fever, myalgia,
arthralgia, and peripheral neuropathy.

Ha H K et al. Radiographics 2000;20:779-794

©2000 by Radiological Society of North America

 Figure 8a.  Polyarteritis nodosa with bowel ischemia in a 58-year-old man.

Rha S E et al. Radiographics 2000;20:29-42

©2000 by Radiological Society of North America

 Figure 8b.  Polyarteritis nodosa with bowel ischemia in a 58-year-old man.

Rha S E et al. Radiographics 2000;20:29-42

©2000 by Radiological Society of North America

 Figure 8c.  Polyarteritis nodosa with bowel ischemia in a 58-year-old man.

Rha S E et al. Radiographics 2000;20:29-42

©2000 by Radiological Society of North America

SLE
 Involves small- and medium-sized vessels
 Involves GI tract in up to 50 percent of patients.
 Pt can present with intermittent lower abdominal pain
for months prior to the development of an acute
abdomen
 Other sx includes nausea, vomiting, diarrhea, GI
bleeding, and fever secondary to mesenteric vasculitis
 Risk factors for the development of mesenteric vasculitis
- peripheral vasculitis and central nervous system lupus.
- hx of mesenteric thrombosis and infarction (esp with
positive antiphospholipid antibodies )
 In an older series published in 1982
◦ 15 of 140 patients with SLE who required hospital
admission developed a disease-related abdominal event
◦ 11 underwent exploratory laparotomy
◦ 6 intestinal perforations (5 colonic, 1 in the small
intestine)
◦ 3 had impending bowel perforation
◦ 4 patients responded to corticosteroids alone while
surgery was being considered
 Current therapy of severe SLE is more aggressive
and typically consists of intravenous pulse
methylprednisolone and pulse cyclophosphamide
 The majority of patients in this study have
had a prodrome sx
◦ insidious onset of intermittent, lower quadrant
cramping and abdominal pain, which was present
for an average of 34 days prior to hospitalization
◦ At presentation : anorexia, nausea, and vomiting
were common(in addition to abdominal pain)
5 patients (33 percent) had diarrhea and melena
Antiphospholipid syndrome
 due to ischemia 2o to thrombosis or
thromboembolism involving the esophagus,
stomach, duodenum, jejunoileum, or colon
 Causes:

◦ GI bleed
◦ abdominal pain
◦ acute abdomen
◦ esophageal necrosis with perforation
◦ giant gastric ulceration
Why we need to differentiate?
  APS is not a vasculitis
 When abdominal symptoms occur in a patient

with the APS and SLE, the patient may

erroneously be considered to have a
mesenteric vasculitis
 treatment of the APS consists of

anticoagulation, not immunosuppressive

therapy
 Dx of the APS is made by consideration of both the clinical
history and the laboratory findings, particularly in patients
with SLE. Criteria :
- at least 1 of the following clinical findings
- at least 1 of the following laboratory test results which must
be positive on at least two occasions more than three months

 Clinical
◦ Recurrent venous or arterial thrombosis, recurrent fetal loss,
persistent thrombocytopenia, and/or livedo reticularis
 Laboratory
◦ IgG or IgM anticardiolipin antibody, anti-ß2-glycoprotein I antibody,
and/or lupus anticoagulant activity
 Figure 7a.  Systemic lupus erythematosus with mesenteric ischemia in a 20-year-old man.

Rha S E et al. Radiographics 2000;20:29-42

©2000 by Radiological Society of North America

 Figure 7b.  Systemic lupus erythematosus with mesenteric ischemia in a 20-year-old man.

Rha S E et al. Radiographics 2000;20:29-42

©2000 by Radiological Society of North America

 Figure 9a.  Systemic lupus erythematosus in a 37-year-old woman who presented with fever,
cough, abdominal pain, polyarthralgia, and skin rash.

Ha H K et al. Radiographics 2000;20:779-794

©2000 by Radiological Society of North America

 Figure 9b.  Systemic lupus erythematosus in a 37-year-old woman who presented with fever,
cough, abdominal pain, polyarthralgia, and skin rash.

Ha H K et al. Radiographics 2000;20:779-794

©2000 by Radiological Society of North America

 Figure 10a.  Systemic lupus erythematosus in a 26-year-old woman who complained of
sudden onset of abdominal pain, diarrhea, and vomiting and met the clinical diagnostic
criteria for this disorder.

Ha H K et al. Radiographics 2000;20:779-794

©2000 by Radiological Society of North America

 Figure 10b.  Systemic lupus erythematosus in a 26-year-old woman who complained of
sudden onset of abdominal pain, diarrhea, and vomiting and met the clinical diagnostic
criteria for this disorder.

Ha H K et al. Radiographics 2000;20:779-794

©2000 by Radiological Society of North America

Take home message
 Affects small to medium vessel
 Common – PAN
 Suspect if severe abdominal pain out of

proportion to clinical assessment

 CT abdomen best imaging
 Rx - intravenous pulse methylprednisolone

and pulse cyclophosphamide, surgery

 Differentiate APS – as Rx is anticoagulation

not immunosuppressives
Thank you