vasculitis affecting git

1. GIT Vasculitis
Prof.Adel Abd Eslam
Professor of internal medicine
Rheumatology&immunology unit

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
perforation.
• Bowel ischemia and perforation are associated with
significant mortality.
• Clinical suspicion must confirmed radiology by CT and
endoscopic histological biopsy.
Hokama A et al,2012;
GIT Vasculitis

5. GIT is primary site of vasculitis;

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
steroid.

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
both diseases.

9. Medium vessel vasculitis;
Kawasaki disease;
• Vasculitis involving large, medium and small
arteries.
• Associated with mucocutaneous lymph node
syndrome.
• Usually in children, coronary arteries are
involved.
• GIT is relatively uncommon, but acute abdomen
with paralytic ileus, ischemic enteritis and
vasculitic appendicitis may occur.
Morgan MD,etal2005

10. polyarteritis-nodosa;
• 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
media.
• Typical radiological feature include; aneurysm
up to 1 CM in diameter within renal,
mesenteric and hepatic vasculature.
polyarteritis-nodosa;

13. ANCA associated vasculitis;
Eosinophilia granulmatosis with polyangitis / churg-stauss
syndrome.
• 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.
GIT manifestation;
• (age 40 to 60), male > female.
• Abdominal pain and bloody diarrhea are two most characteristics
symptoms.
• When mesenteric vasculitis due to EGPA, histological finding similar
to PAN.

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.
GIT manifestation;
• 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
vasculitis)

17. Granulomatosis polyangitis/ Wegner granulomatosis;
• More in middle age, may all age.
• No gender difference.
• Vasculitis with granuloma and large area of vessel
necrosis.
• Small and medium sized arteries and veins.
• Any vessel from anywhere of body can be affected,
with predication for upper respiratory tract, lung and
kidney.
• 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
cascade.
• Children> adult, self limited in children, very poor prognosis in
adult.
• 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,
hematochesia.
• 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
cryoglobulin deposition.
• Either related to HCV ‘HCV cryoglobulinemic’,or
idiopathic.
• 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
dchronic infections.
• Type2 and 3 are related to vasculitis.

22. SLE;
• Defective T cell/ activate B cell, deposition C3 and
immune complex in the media and adventitia,
necrotizing vasculititis.
• 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
pesudobstruction.
• 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.

28. Akira Hokama,,etal2012

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
diffuse.
Localized lesion
• cause deeply penetrating ulcer in ileocecal valve with high
frequency of hemorrhage.
• CT, mass like lesion and thickened bowel with marked
enhanced lesion.
• Barium, marked irregular ulcer with marked thickening of
intestinal wall.
Diffuse lesion;
• 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.
• SOV can occur in anywhere in the body, several location inside
abdomen.
• 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 ,
asymptomatic.
• 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.
http://dx.doi.org/10.1016/j.pathol.2017.05.013

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
presentation.

38. Conclusion:
• 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.

39. Reference:
• 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.