2. • It can affect vessel of all size.
• The clinical course and pathological features are quite
variable dependent on size and location of affected vessel.
• It can cause local or diffuse pathologic changes in the GIT.
• Clinical features; ulcer, submucosal edema, hemorrhage,
paralytic ileus, mesenteric ischemia, bowel obstruction and
• Bowel ischemia and perforation are associated with
• Clinical suspicion must confirmed radiology by CT and
endoscopic histological biopsy.
Hokama A et al,2012;
6. DD of GIT vasculitis;
1. CMV vasculitis:
• GIT symptoms are non specific, abdominal pain,
diarrhea, GI bleeding.
• The colon and stomach.
• Endoscopic feature; normal mucosa, diffuse erythema,
nodule, pseudotumor, erosion and ulcer.
• Pathologic proof of classic intranuclear inclusion body
not always possible due to infection of vascular
endothelium, connective stroma under the ulcer.
• PCR of the biopsy, CMV antigemia assay help diagnosis.
• Most cases respond to ganciclovir.
7. 2. NSAID induced GI damage;
Various type of enteropathy in small and large
intestine due to long stand NSAID.
Gastrodudoneal peptic ulcer has been reported.
Improvement symptoms with discontuation of
8. Manifestation of GIT vasculitis;
Large vessel vasculitis:
1. Gaint cell arteritis;
• Granulomatus arteritis of the aorta and its major branches with
predilection to cranial branches of the cartoid artery .
• The frequency of GIT involvement is very rare.
2. Takayasus arteritisi;
• Granulomatus inflammation of the aorta and its major branches
with characteristic occular affection and decrease brachial artery
pulse (pulseless disease).
• The descending aorta syndrome cause mesenteric vasculitis, but
the frequency of mesenteric and celiac involvement is rare.
• Farrant M,etal2008 reported a young women female develop TA
following Crohn disease , concluded that common pathology in
9. Medium vessel vasculitis;
• Vasculitis involving large, medium and small
• Associated with mucocutaneous lymph node
• Usually in children, coronary arteries are
• GIT is relatively uncommon, but acute abdomen
with paralytic ileus, ischemic enteritis and
vasculitic appendicitis may occur.
• Medium sized vessel.
• Usually spare veins.
• Necrotizing inflammation without glomerulonephritis.
• Fourth decade of life.
• Male> female by 2 folds.
• ANCA negative.
• HBV, HCV, drug abuser, idiopathic.
• Usually vessel in the skin, kidney, nerves and GIT.
• GIT; 50%, acute, abdominal pain, diarrhea, bleeding,
hemorrhage, infarction, peritonitis, cholecystitis,
infarction, hematochezia and perforation.
11. • Mimic inflammatory bowel disease.
• Pathology; trans mural necrosis, fibrinoid
necrosis, all layers are involved, centered to
• Typical radiological feature include; aneurysm
up to 1 CM in diameter within renal,
mesenteric and hepatic vasculature.
13. ANCA associated vasculitis;
Eosinophilia granulmatosis with polyangitis / churg-stauss
• Least common.
• Vasculitis of small/medium vessel.
• Eosinophilia rich >10%/ necrotizing granulomatous.
• Sever asthma, fever, eosinophilia, cardiac, renal failure, peripheral
neuropathy, pulmonary infiltrate, sinusitis, purpura, subcutaneous
nodule and hypertension.
• (age 40 to 60), male > female.
• Abdominal pain and bloody diarrhea are two most characteristics
• When mesenteric vasculitis due to EGPA, histological finding similar
14. • DD; all causes of eosinophilia and vasculitis;
Idiopathic hypereosinphiiic syndrome, parasite,
hypersensitivity drugs, GPA, MPA.
1. The vasculitis is centered to small vessel,
capillaries, venule/arterioles, rarely to medium
sized arteries and veins.
2. Perivascular eosinophilia infiltration,
arteriopathy > true vasculitis.
3. Epithelioid and giant cells are found around
vessel, extravascular eosinophilia rich
granuloma, that spills over surrounding tissue.
16. Microscopic polyangitis (MPA):
• Microscopic form of PAN, hypersensitivity vasculitis.
• 1994, it is a disease entity, despite overlap with PAN, EGPA, GPA,
cutaneous leukocytoclastic vasculitis.
• All ages, all ethnicity, equal male and female.
• Involve small vessel mainly, occasionally medium sized vessel.
• Rarely affected, abdominal pain is the most common symptoms.
• The paucity of immune complex in MPA differentiated it from GPA,
EGPA by absence granuloma, peripheral eosinophilia, asthma.
• DD; immune complex mediated vasculitis (HSP, cryoglobulinemia
17. Granulomatosis polyangitis/ Wegner granulomatosis;
• More in middle age, may all age.
• No gender difference.
• Vasculitis with granuloma and large area of vessel
• Small and medium sized arteries and veins.
• Any vessel from anywhere of body can be affected,
with predication for upper respiratory tract, lung and
• GIT involvement is extremely rare.
18. Immune complex vasculitis;
Henoch- schonlein purpura;
Ig A vasculitis; (IgAV):
• Linked to immunization, food allergy, infection(GABHS),
drugs(quinine, ranitidine, clarithromycin).
• Ig A glycosated, recruit inflammatory mediators, complement
• Children> adult, self limited in children, very poor prognosis in
• Systemic or localized (kidney).
• Skin (purpura), joint (arthritis), kidney (nephritis), GIT.
• Leukoctoclastic vasculitis of small and medium capillaries, vein
and arteries, with deposition of IgA and C3.
• DD; MPA, GPA, EGPA, leukocyotoclastic vasculitis.
19. GIT manifestation;
• In 50-85% of IgA vasculitis, 14% first presentation.
• Any part of GIT, second part of the dueodenum and
terminal ileum are preferentially.
• Diarrhea, distension, colicky abdominal pain caused by
bowel ischemia and edema.
• serious complication include; obstruction,
intususception, perforation, hematmesis,
• Endoscopic feature; diffuse mucosal redness,
petechiae, hemorrhagic erosion and ulcer.
• CT abdomen; bowel thickening with the target sign and
engorgement of mesenteric vessel with comb sign.
21. Cryoglobulinemic vasculitis;
• Small vessel, capillries, venules, arterioles with
• Either related to HCV ‘HCV cryoglobulinemic’,or
• 3 types;
1. Type1; monoclonal, lymphoma, waldastrom , meyloma.
2. Type2; monoconal, RF related, lymphoproliferative,
rheumatic diseases, chronic infection.
3. Type3; polyclonal, RF related, rheumatic disease an
• Type2 and 3 are related to vasculitis.
• Defective T cell/ activate B cell, deposition C3 and
immune complex in the media and adventitia,
• Small and medium vessel arteritis/venulitis with
fibrinoid necrosis in sever cases.
• GIT manifestation;
• Any part, liver and pancreas.
• Ischemic changes affect any layer of GIT;
• Mucosa;;;;;; ulcer and hemorrhage.
• Submucosa;;;;;;; edema.
• Muscularis mucosa;;;;;;;;;;;; intestinal
• Serosa;;;;;;;;;;;ascitis and perforation.
27. • CT abdomen; focal or diffuse wall thickening with the target
sign, bowel dilatation, ascitis and engorgment of
mesenteric vessel with comb sign
• Endoscopic biopsy must obtained from deep part of
intestinal wall to confirm the diagnosis.
• Lupus mesenteric vasculititis (LMV).
• LMV rarely cause pneumonitis intestinalis (PI), which may
cause hepatic venous gas with high mortality rate.
• Protein loosing enteropathy, lymphangiectasia which may
caused by immunological vascular or mucosal damage.
• Peritonitis, pancreatitis, GIT vasculitis and abdominal pain.
29. • Behcets syndrome:
• Non sepefic necrotizing vasculitis.
• Triad of oral and genital ulcer, uveitis.
• GIT is integral part of behcet with characteristic ileocecal
valve and esophagus.
• Behcets characterized by ulcerating lesion, either localized or
• cause deeply penetrating ulcer in ileocecal valve with high
frequency of hemorrhage.
• CT, mass like lesion and thickened bowel with marked
• Barium, marked irregular ulcer with marked thickening of
• Multiple discrete punched out ulcer, mainly in the colon.
31. Mixed connective tissue disease;
• SLE, scleroderma and Polymyositis overlap symptoms.
• antiU1 ribonucleoprotein antibodies.
• Dysphagia, heartburn, perforation and malabsorption.
Drug induced vasculitis;
• Either with first use or with long period.
• All pharmacological drugs cause drug induced
vasculitis/lupus like syndrome.
• Cutaneous vasculitis in majority after 7-12 days of
administration, systemic vasculitis in minority.
32. GIT manifestation;
• Involved as part of systemic vasculitis rather than isolated
manifestation or very rarely initial presentation.
• diagnosis depend on temporal relation between clinically
evident vasculitis and drug administration.
• Most cases require stoppage offending drugs, in minority
of cases resistant and require immunosuppresion.
• Clinical presentation; nonspecific, similar to primary
vasculitis, most case are restricted to single organ , but
involvement of kidney, skin, lung .
• Usually good prognosis,
33. Single organ vasculitis
• Isolated PAN like vasculitis.
• arteries, veins of any size.
• a single organ with no evidence of systemic vasculitis.
• Unifocal or multifocal within an organ.
• It is a limited expression of systemic vasculitis process.
• Clinical, serological and pathological correlation is required to
confirm the diagnosis systemic vasculitis limited in single organ;
• SOV can occur in anywhere in the body, several location inside
• GIT is the most frequently site of SOV.
• Any part of GIT, esophagus stomach, omentum, Small and large
intestine, appendix, gall bladder, pancreas.
34. • GIT symptoms;
• pain, hemorrhage, infarction.
• or purely incidental ,
• or occult with the patient
presenting with symptoms
unrelated to vasculitis.
• Incidental or isolated vasculitis of female genital
tract is addressed(Abu-Farsakh H;etal1994).
• PAN like or giant cell arteritis affecting uterine
cervix and myometrium.
35. Relation of SOV to systemic vasculitis;
• Not addressed a specific relationship.
• Case report; a true incidental SOV and need to be
managed, does not report a temporal relationship.
• Every SOV case should be flagged, work up to exclude
latent or dormant systemic vasculitis , don’t need
treatment , only routine follow up.
• Follow up preoid is not uniformly agreed but
Gonzalez-Gay and colleagues recommended, a full
work up for 3-5 years would be sufficient.
• Association with vasculitis is not known, the
possibility of some unknown, undetected antigen or
local factor could be operative.
36. • Hoppé et al described 99.1% of SOV with no
progression to systemic vasculitis.
• By this mean , the vasculitis was unsuspected,
asymptomatic and specimen harboured other
pathology which was the reason for removal.
• Surgical pathologist are akin to examine the
vasculture even when the primary diagnosis
explain patients symptoms.
• Communication with clinical colleagues and
clinical pathologist is only way for proper work
up to exclude systemic vasculitis at time of
• Systemic vasculitis can initially manifest in the GIT.
• Histological examination for evidence of vasculitis
(hemorrhage, infarction and ischemia) especially in
the omentum and deep layer of the bowel.
• If highly clinical suspicious of vasculitis without
evidence of histological finding, multiple section
should be examined.
• SOV without evidence of systemic vasculitis, can be
encounted co-existing GI vasculitis.
• Gonzalez-Gay MA, Vazquez-Rodriguez TR, Miranda-Filloy JA, Pazos-Ferro A, Garcia-
Rodeja E. Localized vasculitis of the gastrointestinal tract: a case report and
literature review. Clin Exp Rheumatol 2008; 26(Suppl 49): S102e5.
• Abu-Farsakh H, Mody D, Brown RW, Truong LD. Isolated vasculitis involving the
female genital tract: clinicopathologic spectrum and phenotyping of inflammatory
cells. Mod Pathol 1994; 7: 610e5.