Gastrointestinal Scleroderma Imaging
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Gastrointestinal scleroderma is the third most common manifestation of scleroderma, or systemic sclerosis (SSc), after skin changes and Raynaud phenomenon. [1, 2] GI symptoms are the first manifestation of SSc in nearly 10% of cases. [3] 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. [4, 2, 5, 6, 7, 8, 9, 10, 11, 12]
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. [4]
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. [4]
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. [4]
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. [4]
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. [4]
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. [4]
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. [13] These complications are associated with recurrent hospitalizations, and malabsorption is an independent predictor of mortality. [14] Acute GI complications such as mechanical or pseudo-obstruction can be life-threatening in the context of other organ based involvement, especially cardiorespiratory disease. [4]
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. [15] 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. [16] 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.
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. [17, 9, 12, 18]
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. [13]
Use of endoluminal ultrasonography to define transmural pathology is increasing. Ultrasonography is also useful in gastric-emptying studies. [19] 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. [20]
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.
Conventionally, radiography is usually used to detect musculoskeletal involvement, calcinosis, pulmonary fibrosis, and pericardial effusion. Clinical pericarditis is present in only 10-15% of patients with scleroderma and is more common in patients with limited disease. Pericarditis may mimic symptoms of esophageal involvement, and lung fibrosis often accompanies gastrointestinal scleroderma. [21, 22]
Gastric atony and dilation may occur, but involvement of the stomach is uncommon compared with involvement of other portions of the gastrointestinal tract. In rare instances, telangiectasia is the source of serious bleeding from the distal esophagus, stomach, or other gastrointestinal tract sites, especially in persons with limited cutaneous disease. All of these features may be depicted on an upper GI barium series, though the sensitivity of barium study is low in telangiectasia.
A high percentage of patients with connective tissue disease have significant esophageal motility disorders. Investigations are more likely to be positive with the CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) than with other connective tissue diseases, even if dysphagia is present. Barium meal study and manometry are more useful than esophagoduodenoscopy. The sensitivity of esophagography for all manometric esophageal disorders is 56%, and its specificity is 91%. However, when nonspecific esophageal motility is excluded, the sensitivity rises to 89%. [17]
Esophageal involvement may mimic achalasia and esophageal peptic ulceration. Small bowel dilation may mimic small bowel obstruction. Large bowel dilation may mimic Hirschsprung disease. Gas may be released into the peritoneum from pneumatosis intestinalis and may mimic a perforated abdominal viscus.
The following findings may be observed in colonic scleroderma (see the images below):
Large bowel constipation is common and may be seen on conventional radiographs or a barium enema study.
In rare cases, diarrhea is noted, but more frequently, constipation and lower abdominal distention or fecal impaction manifestations of colonic involvement are present.
Patents with scleroderma have decreased distensibility of the colon that is not necessarily correlated with symptoms.
Large-mouthed pseudodiverticula are seen on the antimesenteric border, mostly in the transverse and descending colon.
In advanced disease, the haustra are effaced.
The colon may be markedly dilated.
Stercoral ulceration may complicate colonic scleroderma.
Reduced anorectal motility, compliance, and sphincter pressure are reported; rectal incontinence and prolapse are uncommon but disabling problems; these complications may be imaged by using a barium defecogram.
Contrast-enhanced studies (barium series) are generally used in the investigation of gastrointestinal involvement in scleroderma. The esophagus is studied by means of barium swallow study, which may show the following results:
Hypotonia and diminished peristalsis in the lower two thirds of the esophagus
Esophageal activity in the upper third is usually normal, as it contains striated muscle
Loss of the mucosal longitudinal folds
Mild to moderate esophageal dilatation
Patulous appearance of the lower esophageal sphincter
Impaired emptying of the esophagus with the patient in the recumbent position
Gastroesophageal reflux (elicited in >70% of patients)
Erosions and superficial ulcers in the lower esophagus may be associated with gastroesophageal reflux
Fusiform stricture 4-5 cm above the gastroesophageal border secondary to reflux esophagitis
Barrett esophagus, often associated with gastroesophageal reflux, may involve a long esophageal stricture in the mid and/or lower esophagus, a large and deep wide-mouthed esophageal ulcer, and a fine reticular mucosal pattern distal to the stricture
In scleroderma, small bowel disease occurs in up to 45% cases and is usually a poor prognostic indicator. The disease process is rapidly progressive once the small bowel is involved. The mainstay of small bowel imaging remains barium examination, which may show the following (see the images below):
A malabsorption pattern may occur with the fragmentation/flocculation of barium
Excessive fluid may be observed in the small bowel, which is due to bowel dilation and bacterial overgrowth causing barium dilution and poor coating
Transit time may be delayed, with barium retention in the duodenum for up to 24 hours
Marked dilatation of small bowel may give rise to megaduodenum or megajejunum, which mimics small bowel obstruction
An abrupt cutoff may be noted at the level of the superior mesenteric artery because of atrophy of the neural cells with hypoperistalsis
Tightly packed mucosal folds may be present in the hidebound pattern, or accordion pattern
The mucosal folds may be normal despite histologic evidence of disease
The study may show asymmetric pseudodiverticulae/sacculations with squared tops with broad bases on the mesenteric side
Occasionally, pneumatosis intestinalis occurs in patients with advanced bowel disease; in this situation, intestinal gas dissects into the bowel wall or into the peritoneal cavity, mimicking a perforated bowel
Using cineradiography, Campbell and Schultz found that abnormal peristalsis (absent or diminished) was 67% sensitive for scleroderma. Although a high sensitivity value (87%) for cineradiography can be obtained by considering any peristaltic abnormality as a sign of scleroderma, this is achieved at the price of an undesirably high number of false-positive results (specificity, 40%)
Computed tomography is generally not used to evaluate gastrointestinal involvement, but it may be indicated in staging esophageal cancer, in assessing intestinal obstruction and bowel perforation, and in establishing the differential diagnosis of primary biliary cirrhosis associated with scleroderma.
Pneumatosis cystoides intestinalis (PCI) is a rare but life-threatening gastrointestinal complication in the course of scleroderma-related conditions. PCI is characterized by the appearance of intramural clusters of gas in the small and large bowel wall on plain radiographs and/or CT scans and is often accompanied by a pneumoperitoneum. One study concluded that recognition of PCI is necessary for appropriate therapeutic application. In patients with underlying scleroderma-related conditions and symptoms of abdominal emergency, recruitment of multidisciplinary teams, including a rheumatologist, gastroenterologist, imaging specialist, and surgeon familiar with intestinal complications of scleroderma-related conditions, is warranted. [24]
CT is the examination of choice in alveolitis and pulmonary fibrosis associated with scleroderma. CT is also a sensitive means of differentiating various causes of jaundice and/or abnormal liver function test results.
Ultrasonography is an extremely sensitive means of differentiating various causes of jaundice in the context of primary biliary cirrhosis associated with scleroderma and is a sensitive means of screening patients presenting with abdominal pain in association with scleroderma. It is increasingly being used for cross-sectional imaging of the bowel. Ultrasonography remains operator dependent, and images can be degraded by fat, patient habitus, gas, and bones. Therefore, a potential for false-positive or false-negative findings always exists. [19]
Motility disorders of the gallbladder seem to play a minor role in upper GI tract involvement in systemic sclerosis, whereas motility disorders of the stomach are frequent and can be easily recognized on noninvasive real-time ultrasonography. [25]
The development of ultrasound probes based on 6F to 10F catheters has made high-resolution endoluminal ultrasonography available for use in a variety of lumina. Endoluminal ultrasonography with frequencies of 9-20 MHz has been used for evaluation of a wide range of abnormalities, including esophageal varices, various submucosal lesions, and fibrosis in scleroderma. This technique may expand diagnostic capabilities of ultrasonography, providing important information for decision making relative to patient care and minimally invasive interventional procedures. [26]
Radionuclide esophageal transit testing is a safe and noninvasive method that is more sensitive than cineradiography. This method might be used as a screening test to evaluate esophageal involvement in patients with progressive scleroderma. [27]
Wojas-Pelc et al performed esophageal scintigraphy in 30 patients with scleroderma to define the relationship between subjective symptoms of the esophagus and results of esophageal scintigraphy. Independent of esophageal dysmotility symptoms, which were noted by patients, esophageal scintigraphy registered changes in 97% of the patients. The most common esophageal dysmotility was observed in the lower part of the esophagus. Statistically, dysmotility of the central portion of the esophagus was observed more often in patients with diagnosed scleroderma than in others. [28]
Technetium-labeled RBCs or technetium sulfur colloid scanning is often used as an initial screening tool in diagnosing the site of gastrointestinal bleeding. The sensitivity of labeled RBC for gastrointestinal hemorrhage is 93%, and the specificity is 95%. The sensitivity of sulfur colloid for the bleeding site is only 12%, but it has a specificity of 100%.
Scintigraphy can provide a sensitive quantitative index of esophageal dysmotility. In scleroderma, the esophageal clearance of a semisolid test meal is well correlated with the results of barium studies, manometry, and ultrasonography. The severity of associated gastroesophageal reflux can also be reliably assessed.
Hansi N, Thoua N, Carulli M, Chakravarty K, Lal S, Smyth A, et al. Consensus best practice pathway of the UK scleroderma study group: gastrointestinal manifestations of systemic sclerosis. Clin Exp Rheumatol. 2014 Nov-Dec. 32 (6 Suppl 86):S-214-21. [Medline].
Dalle I, Geboes K. Vascular lesions of the gastrointestinal tract. Acta Gastroenterol Belg. 2002 Oct-Dec. 65 (4):213-9. [Medline].
McFarlane IM, Bhamra MS, Kreps A, Iqbal S, Al-Ani F, Saladini-Aponte C, et al. Gastrointestinal Manifestations of Systemic Sclerosis. Rheumatology (Sunnyvale). 2018. 8 (1):235. [Medline]. [Full Text].
Shreiner AB, Murray C, Denton C, Khanna D. Gastrointestinal Manifestations of Systemic Sclerosis. J Scleroderma Relat Disord. 2016. 1 (3):247-256. [Medline]. [Full Text].
Della Rossa A, Valentini G, Bombardieri S, et al. European multicentre study to define disease activity criteria for systemic sclerosis. I. Clinical and epidemiological features of 290 patients from 19 centres. Ann Rheum Dis. 2001 Jun. 60 (6):585-91. [Medline].
Haustein UF. Systemic sclerosis-scleroderma. Dermatol Online J. 2002 Jun. 8 (1):3. [Medline].
LeRoy EC, Medsger TA Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol. 2001 Jul. 28 (7):1573-6. [Medline].
Lonzetti LS, Joyal F, Raynauld JP, Roussin A, Goulet JR, Rich E, et al. Updating the American College of Rheumatology preliminary classification criteria for systemic sclerosis: addition of severe nailfold capillaroscopy abnormalities markedly increases the sensitivity for limited scleroderma. Arthritis Rheum. 2001 Mar. 44 (3):735-6. [Medline].
Schmults CA. Scleroderma. Dermatol Online J. 2003 Oct. 9 (4):11. [Medline].
Domsic R, Fasanella K, Bielefeldt K. Gastrointestinal manifestations of systemic sclerosis. Dig Dis Sci. 2008 May. 53 (5):1163-74. [Medline].
Di Ciaula A, Covelli M, Berardino M, Wang DQ, Lapadula G, Palasciano G, et al. Gastrointestinal symptoms and motility disorders in patients with systemic scleroderma. BMC Gastroenterol. 2008 Feb 27. 8:7. [Medline].
Chapin R, Hant FN. Imaging of scleroderma. Rheum Dis Clin North Am. 2013 Aug. 39 (3):515-46. [Medline].
Miller JB, Gandhi N, Clarke J, McMahan Z. Gastrointestinal Involvement in Systemic Sclerosis: An Update. J Clin Rheumatol. 2018 Sep. 24 (6):328-337. [Medline]. [Full Text].
Codullo V, Cereda E, Klersy C, Cavazzana I, Alpini C, Bonardi C, et al. Serum prealbumin is an independent predictor of mortality in systemic sclerosis outpatients. Rheumatology (Oxford). 2016 Feb. 55 (2):315-9. [Medline].
Alastal Y, Hammad TA, Renno A, Khalil B, Pierre J, Kwaah B, et al. Gastrointestinal manifestations associated with systemic sclerosis: results from the nationwide inpatient sample. Ann Gastroenterol. 2017. 30 (5):498-503. [Medline]. [Full Text].
Denaxas K, Ladas SD, Karamanolis GP. Evaluation and management of esophageal manifestations in systemic sclerosis. Ann Gastroenterol. 2018 Mar-Apr. 31 (2):165-170. [Medline]. [Full Text].
Ott DJ, Richter JE, Chen YM, et al. Esophageal radiography and manometry: correlation in 172 patients with dysphagia. AJR Am J Roentgenol. 1987 Aug. 149(2):307-11. [Medline].
Lidia Vera-Lastra O. [Systemic sclerosis and the gastrointestinal tract. Diagnostic and therapeutic approach]. Reumatol Clin. 2006 Nov. 2 Suppl 3:S24-30. [Medline].
Muresan C, Surdea Blaga T, Muresan L, Dumitrascu DL. Abdominal Ultrasound for the Evaluation of Gastric Emptying Revisited. J Gastrointestin Liver Dis. 2015 Sep. 24 (3):329-38. [Medline].
Zuccaro G. Esophagoscopy and endoscopic esophageal ultrasound in the assessment of esophageal function. Semin Thorac Cardiovasc Surg. 2001 Jul. 13(3):226-33. [Medline].
Follansbee WP, Curtiss EI, Rahko PS, et al. The electrocardiogram in systemic sclerosis (scleroderma). Study of 102 consecutive cases with functional correlations and review of the literature. Am J Med. 1985 Aug. 79(2):183-92. [Medline].
Follansbee WP. The cardiovascular manifestations of systemic sclerosis (scleroderma). Curr Probl Cardiol. 1986 May. 11(5):241-98. [Medline].
Wipff J, Coriat R, Masciocchi M, Caramaschi P, Derk CT, Hachulla E, et al. Outcomes of Barrett’s oesophagus related to systemic sclerosis: a 3-year EULAR Scleroderma Trials and Research prospective follow-up study. Rheumatology (Oxford). 2011 Aug. 50 (8):1440-4. [Medline].
Balbir-Gurman A, Brook OR, Chermesh I, Braun-Moscovici Y. Pneumatosis cystoides intestinalis in scleroderma-related conditions. Intern Med J. 2012 Mar. 42(3):323-9. [Medline].
Wedmann B, Wegener M, Adamek RJ, el Gammal S. Gastrobiliary motility after liquid fatty meal in progressive systemic sclerosis. A sonographic study. Dig Dis Sci. 1994 Mar. 39(3):565-70. [Medline].
Liu JB, Miller LS, Bagley DH, Goldberg BB. Endoluminal sonography of the genitourinary and gastrointestinal tracts. J Ultrasound Med. 2002 Mar. 21(3):323-37. [Medline].
Wang SJ, Lan JL, Chen DY, et al. Solid-phase radionuclide esophageal transit in progressive systemic sclerosis. Hepatogastroenterology. 2002 Jul-Aug. 49(46):989-91. [Medline].
Wojas-Pelc A, Stepien A, Lipko-Godlewska S, et al. Esophageal scintigraphy in patients with systemic sclerosis: clinical symptoms correlated with the esophagus noted by the patients [in Polish]. Przegl Lek. 2002. 59(12):973-6.
Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR Consultant Radiologist and Honorary Professor, North Manchester General Hospital Pennine Acute NHS Trust, UK
Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR is a member of the following medical societies: American Association for the Advancement of Science, American Institute of Ultrasound in Medicine, British Medical Association, Royal College of Physicians and Surgeons of the United States, British Society of Interventional Radiology, Royal College of Physicians, Royal College of Radiologists, Royal College of Surgeons of England
Disclosure: Nothing to disclose.
Rumana Rahim, MBBS, MRCS, FRCR Consultant Radiologist, Kings College Hospital, Denmark Hill, London, UK
Disclosure: Nothing to disclose.
Eugene C Lin, MD Attending Radiologist, Teaching Coordinator for Cardiac Imaging, Radiology Residency Program, Virginia Mason Medical Center; Clinical Assistant Professor of Radiology, University of Washington School of Medicine
Eugene C Lin, MD is a member of the following medical societies: American College of Nuclear Medicine, American College of Radiology, Radiological Society of North America, Society of Nuclear Medicine and Molecular Imaging
Disclosure: Nothing to disclose.
Arnold C Friedman, MD, FACR Professor, Department of Radiology, University of Florida Health Science Center; Chief, Department of Radiology, Shands-Jacksonville Hospital
Arnold C Friedman, MD, FACR is a member of the following medical societies: American College of Radiology, American Institute of Ultrasound in Medicine, American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.
Sumaira MacDonald, MBChB, PhD, MRCP, FRCR Lecturer, Sheffield University Medical School; Endovascular Fellow, Sheffield Vascular Institute
Sumaira MacDonald, MBChB, PhD, MRCP, FRCR is a member of the following medical societies: British Medical Association, Royal College of Physicians, and Royal College of Radiologists
Disclosure: Nothing to disclose.
Eric P Weinberg, MD Associate Professor, Department of Radiology, University of Rochester Medical Center, Strong Memorial Hospital
Eric P Weinberg, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, and Radiological Society of North America
Disclosure: Nothing to disclose.
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