journalclub rheumato 2011

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


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.


3/36


4/36


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.


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.


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


8/36

` 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


9/36


10/36

` vasculitis-like hemorrhage, small ulcers and

edema of the duodenum


11/36


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


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


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


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)


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.


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


18/36

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





19/36

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





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



21/36

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




22/36

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




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 )


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


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


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


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


28/36


29/36

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




30/36

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




31/36


32/36

Figure 9b. Systemic lupus erythematosus in a 37-year-old woman who presented with fever,

cough, abdominal pain, polyarthralgia, and skin rash.




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.




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.




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


36/36

journalclub rheumato 2011

Documents