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Vascular and collagen disorders 

Vascular and collagen disorders

Vascular and collagen disorders

Graham Neale

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date: 30 March 2017

A wide range of vascular and collagen disorders may affect the gastro-intestinal tract. Most are quite uncommon but presentations are often dramatic with intestinal bleeding or gangrene.

Vascular insufficiency leading to ischaemic damage of the gut may be caused by occlusion of mesenteric vessels by either arterial thrombosis (usually with atheromatous pathology); or venous thrombosis (in thrombophilic conditions); or arterial emboli (as occurs with atrial fibrillation); or diffuse small vessel occlusion. There are four primary syndromes. (1) Acute mesenteric ischaemia—typically manifest as the sudden onset of abdominal pain, initially without marked tenderness or localizing signs. Diagnosis requires a high index of suspicion and is often delayed. Imaging serves primarily to exclude other causes of an apparent abdominal catastrophe but prompt laparotomy for embolectomy and/or arterial re-construction and/or bowel resection is required to minimize damage to the gut. (2) Chronic mesenteric ischaemia—usually due to atheroma, presents with severe cramping abdominal pain 20 to 60 min, after eating. Diagnosis requires vascular imaging and usually the condition can be relieved only by revascularization. (3) Ischaemic colitis—presents with pain and tenderness in the left iliac fossa, nausea and vomiting, followed by the passage of a loose motion containing dark blood. Contrast enema examination characteristically shows ‘thumbprinting’ of the colonic mucosa. Most cases resolve spontaneously so supportive management is usually sufficient. (4) Ischaemia–reperfusion injury—diminishes the barrier function of the gut and may lead to septicaemia in those who are already critically ill.

Vascular malformations in the intestine may present with acute bleeding or an iron deficient anaemia. Conditions include (1) Dieufaloy’s disease—an arterial malformation in the upper stomach that usually presents with recurrent massive bleeding; (2) hereditary telangiectasia—with skin lesions and low grade gastrointestinal bleeding; and (3) angiodysplasia—occurs most commonly in the caecum and ascending colon of older patients and is difficult to diagnose. Discrete vascular malformations may be treated endoscopically, although surgical resection is required in some cases.

Collagen-vascular disorders (systemic sclerosis, systemic lupus erythematosus (SLE), and primary vasculitides) may be associated with damage to the intestine. Abdominal symptoms may occur when the pathological process affects the gut but these rarely dominate the clinical picture.

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