Upload
eida-mohd
View
217
Download
0
Embed Size (px)
Citation preview
8/7/2019 journalclub rheumato 2011
1/36
Rheumatology Journal Club
Gut vasculitis
By Dr Nur Hidayati Mohd Sharif
heumatology Dept Hospital Selayang 11/3/2011
8/7/2019 journalclub rheumato 2011
2/36
` 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-S
trauss syndrome, Henoch-S
chnlein syndrome,systemic lupus erythematosus, rheumatoid vasculitis,
and Behet disease.
8/7/2019 journalclub rheumato 2011
3/36
8/7/2019 journalclub rheumato 2011
4/36
8/7/2019 journalclub rheumato 2011
5/36
` 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.
8/7/2019 journalclub rheumato 2011
6/36
` 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.
8/7/2019 journalclub rheumato 2011
7/36
` For 21 pt with surgical cx10-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 associatedwith increased mortality in multivariate analysisPeritonitis (hazard ratio [HR]= 4.3, p
8/7/2019 journalclub rheumato 2011
8/36
` Abdominal x-ray & U/S can be normal` CT & MRI especially with angiography
bowel wall thickening with or without the target signx alternating layers of high and low attenuation within the thickened
bowel wall due to submucosal edema or hemorrhage
intramural pneumatosis, mesenteric orportal venous gas mesenteric arterial or venous thromboembolism
bowel wall become thinned or occasionally invisible when theinvolved 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
8/7/2019 journalclub rheumato 2011
9/36
8/7/2019 journalclub rheumato 2011
10/36
` vasculitis-like hemorrhage, small ulcers and
edema of the duodenum
8/7/2019 journalclub rheumato 2011
11/36
8/7/2019 journalclub rheumato 2011
12/36
` Other GI or extra-intestinal vasculitis or
angiographic abnormalities (seen in 67% of the 39
patients who underwent angiography) not
predictive of surgical complication orpoor 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
8/7/2019 journalclub rheumato 2011
13/36
` Affects small and medium sized arteries
` 7 % associated with hepatitis B but only 1 percent
ofpatients with HBV developpolyarteritis
develops within 12 months of the initial infection result of deposition of viral antigen-antibody complexes in
vessel walls
1-year survival forpatients with HBV-associated PAN is
lower increased incidence of gastrointestinal bleeding
and perforation
8/7/2019 journalclub rheumato 2011
14/36
` Gastrointestinal involvement occurs in 14-65percent ofpatients with PAN
gallbladder and small bowel most frequent sites
`
The more severe manifestations include :-- intestinal infarction orperforation
- pneumatosis intestinalis
- pseudomembranous colitis
- cholecystitis
8/7/2019 journalclub rheumato 2011
15/36
` Series of 16patients 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 165patients with PAN or Churg-
Strauss syndrome 31 percent of deaths during follow-up were attributable to
gastrointestinal disease (gastrointestinal bleeding orperitonitis
in 11 and pancreatitis in 2)
8/7/2019 journalclub rheumato 2011
16/36
` Rx with corticosteroids and cyclophosphamide -improves survival and symptoms relief
` Forpatients with PAN related to HBV infection orhairy cell leukemia treatment of concurrentdisease is indicated
` Some patients with PAN have chronic GI sx duringinactive period Chronic inflammation of vessels led to progressive
narrowing of the vascular lumens and truearteriosclerosis.
8/7/2019 journalclub rheumato 2011
17/36
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 ofNorth America
8/7/2019 journalclub rheumato 2011
18/36
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 ofNorth America
8/7/2019 journalclub rheumato 2011
19/36
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 ofNorth America
8/7/2019 journalclub rheumato 2011
20/36
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 ofNorth America
8/7/2019 journalclub rheumato 2011
21/36
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 ofNorth America
8/7/2019 journalclub rheumato 2011
22/36
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 ofNorth America
8/7/2019 journalclub rheumato 2011
23/36
` Involves small- and medium-sized vessels` Involves GI tract in up to 50percent ofpatients.` 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 withpositive antiphospholipid antibodies )
8/7/2019 journalclub rheumato 2011
24/36
` In an older series published in 1982
15 of 140patients 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
8/7/2019 journalclub rheumato 2011
25/36
` The majority ofpatients 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)
5patients (33 percent) had diarrhea and melena
8/7/2019 journalclub rheumato 2011
26/36
` 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
8/7/2019 journalclub rheumato 2011
27/36
` 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
8/7/2019 journalclub rheumato 2011
28/36
8/7/2019 journalclub rheumato 2011
29/36
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 ofNorth America
8/7/2019 journalclub rheumato 2011
30/36
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 ofNorth America
8/7/2019 journalclub rheumato 2011
31/36
8/7/2019 journalclub rheumato 2011
32/36
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 ofNorth America
8/7/2019 journalclub rheumato 2011
33/36
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 diagnosticcriteria for this disorder.
Ha H K et al. Radiographics 2000;20:779-794
2000 by Radiological Society ofNorth America
8/7/2019 journalclub rheumato 2011
34/36
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 diagnosticcriteria for this disorder.
Ha H K et al. Radiographics 2000;20:779-794
2000 by Radiological Society ofNorth America
8/7/2019 journalclub rheumato 2011
35/36
` Affects small to medium vessel
` Common PAN
` Suspect if severe abdominal pain out ofproportion
to clinical assessment` CT abdomen best imaging
` Rx - intravenous pulse methylprednisolone and
pulse cyclophosphamide, surgery
` DifferentiateAPS as Rx is anticoagulation notimmunosuppressives
8/7/2019 journalclub rheumato 2011
36/36