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Gastrointestinal Scleroderma Imaging

Practice Essentials

Gastrointestinal scleroderma is the third most common manifestation of scleroderma, or systemic sclerosis (SSc), after skin changes and Raynaud phenomenon.
GI symptoms are the first manifestation of SSc in nearly 10% of cases.
 Scleroderma is a clinically heterogeneous connective tissue disorder that affects the skin; the wall of the blood vessels; and the internal organs, such as the gastrointestinal tract, lungs, heart, and kidneys. This disease is characterized by alterations of the microvasculature, disturbances of the immune system, and massive deposition of collagen and other matrix substances in the connective tissue.

Histopathology of the gastrointestinal tract shows mild inflammatory infiltrates of the lamina propria and, in certain segments, atrophy and fragmentation of the smooth muscles, followed by collagen deposition and changes of the blood vessels. Histologic studies show mainly CD4 T cells, around blood vessels and at sites of active connective tissue formation in scleroderma, emphasizing the role of T lymphocytes in its pathogenesis. Most T-cells are positive for HLA-DR. The ratio of helper CD4+ T lymphocytes relative to suppressor/cytotoxic T lymphocytes is increased in patients with SSc.

Telangiectasias (arteriovenous malformations) can be seen in scleroderma throughout the gastrointestinal tract, including the stomach, small bowel, and colon. Massive gastrointestinal bleeding rarely results from these malformations in scleroderma.

In the gastrointestinal tract, scleroderma can cause progressive atrophy and collagenous fibrous replacement of the muscularis, which may affect any part or all of the gastrointestinal tract but is more prominent in the esophagus. The lower two thirds of the esophagus often develops a rubber hosepipe-like consistency. The mucosa undergoes atrophy, which may be thinned and ulcerated and is often associated with excessive deposition of collagen in the lamina propria and submucosa. Less severe changes generally occur in the walls of the stomach, small bowel, and large bowel. The villi and microvilli atrophy, which is the anatomic basis of malabsorption that is sometimes associated with SSc. The gut vessel walls contain perivascular mononuclear cell infiltrates and hyaline collagenous thickening.

Many patients with scleroderma do not have significant symptoms despite demonstrable abnormalities of gastrointestinal function. Thinning of the lips and reduced oral apparatus are frequent findings. Temporomandibular joint involvement may also limit mouth opening in some patients. Atrophy of the mucous membrane and tongue papilla with impaired taste perception has also been reported.

The gastrointestinal tract is frequently involved in scleroderma (40-45% of cases). The esophagus is involved in more than 85% of cases, with resultant dysphagia and phagodynia due to hypomotility, reflux, peptic esophagitis, Barrett metaplasia, and fibrotic strictures. The stomach is less frequently involved. Abnormalities include decreased motor activity and delayed gastric emptying and gastric dilatation (watermelon stomach). The small bowel is involved in approximately 45% of patients and may affect motility of the small bowel and large bowel, leading to pseudo-obstruction or malabsorption due to bacterial overgrowth. In addition, diverticular ulcerations, stenosis, chronic constipation, megacolon, and rectal prolapse have been described.

Liver involvement is rare and, less often, may be secondary as a sequela of pulmonary heart disease. However, an association with primary biliary cirrhosis has been described. Minor exocrine pancreatic insufficiency may occur and is often of no significance, but it may contribute to malabsorption.

Esophageal involvement may be silent or present with dysphagia and chest pain from reflux and defective esophageal muscular function. Chest pain can be a sign of myocardial/pericardial disease; therefore, investigating the heart becomes an integral part of the clinical management of gastrointestinal scleroderma.

Systemic sclerosis (SSc) is a rare multisystem autoimmune disorder characterized by immune activation, vasculopathy, and abnormal collagen deposition in the skin and various internal organs. The gastrointestinal tract is involved in the limited cutaneous (lcSSc) and diffuse cutaneous (dcSSC) forms of the disease and is present in approximately 90% of patients with SSc.

The severity of GI disease is a marker of worse prognosis and mortality in patients with SSc. Severe GI disease is associated with pseudo-obstruction and malabsorption and may result in dependence on enteral or total parenteral nutrition.
These complications are associated with recurrent hospitalizations, and malabsorption is an independent predictor of mortality.
Acute GI complications such as mechanical or pseudo-obstruction can be life-threatening in the context of other organ based involvement, especially cardiorespiratory disease.

The esophagus is the most common site of GI involvement in SSc patients, with a high prevalence of gastroesophageal reflux disease (GERD), Barrett esophagus, and dysphagia.
Overall, the incidence of esophageal symptoms in SSc has been estimated at between 40 and 80%, even though a percentage of patients are totally asymptomatic despite their documented esophageal disease. Patients with esophageal involvement may report symptoms due to GERD, such as heartburn and regurgitation, and/or symptoms due to esophageal dysmotility, such as dysphagia and chest pain.
Chest pain can be a sign of myocardial/pericardial disease; therefore, investigating the heart becomes an integral part of the clinical management of gastrointestinal scleroderma.

Preferred examination

Conventional radiography is the mainstay of imaging scleroderma (see the radiographic images below). It is used to image the musculoskeletal system, lungs, and abdomen. Plain abdominal images may reveal bowel dilatation, features of constipation, intestinal obstruction, and perforation. Contrast-enhanced studies are generally used to evaluate gastrointestinal involvement in SSc.

Plain abdominal radiograph shows a gas-filled, dil

Plain abdominal radiograph shows a gas-filled, dilated stomach (black arrow) and duodenum (red arrow). Barium-meal study in the same patient shows a dilated duodenum. Note also several diverticulae in the small bowel.

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Plain erect abdominal radiograph shows nonobstruct

Plain erect abdominal radiograph shows nonobstructed dilated loops of bowel with fluid levels. Chest radiograph in the same patient shows basal lung fibrosis.

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Plain erect abdominal radiograph shows nonobstruct

Plain erect abdominal radiograph shows nonobstructed dilated loops of bowel with fluid levels. Chest radiograph in the same patient shows basal lung fibrosis.

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Barium series (see below) should be performed only after careful consideration of the risks and benefits. When barium studies are performed, the patient should be vigorously purged soon thereafter, and follow-up radiographs should be obtained to confirm adequate removal of the barium agent.​

Small bowel barium study shows a dilated duodenum,

Small bowel barium study shows a dilated duodenum, short strictures, and stagnation of the barium agent. Chest radiograph of the same patient shows basal fibrosis.

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Use of endoluminal ultrasonography to define transmural pathology is increasing. Ultrasonography is also useful in gastric-emptying studies.
Because thoracic symptoms in patients with SSc may be related to esophageal dysmotility, pulmonary hypertension, pericarditis, and pulmonary heart disease, echocardiography is an important imaging modality to differentiate between cardiogenic and noncardiogenic symptoms.

Cross-sectional imaging studies, such as CT, are used in assessing thoracic disease and occasionally in the evaluation of abdominal complications of scleroderma. MRI has little if any role to play, though it may be useful in the differential diagnosis of musculoskeletal complications. Angiography may sometimes be required in the diagnosis and treatment of gastrointestinal bleeding. Radionuclide studies have a useful role in assessing esophageal and gastric motility.

Celiac and mesenteric angiography is an accurate means of demonstrating not only the site but also the cause of gastrointestinal bleeding; it also provides the means for therapeutic measures, such as pharmacologic or embolic control of bleeding.

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