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Gastrointestinal
Diagnosis
Small Intestine
Inflammation
Intestinal Scleroderma
Intestinal Scleroderma
Atif Zaheer, MD; Michael P. Federle, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Diagnostic Checklist

          TERMINOLOGY

          • Synonyms

            • Progressive systemic sclerosis

          • Definitions

            • Multisystem disorder of small vessels and connective tissue of unknown etiology

          IMAGING

          • General Features

            • Radiographic Findings

              DIFFERENTIAL DIAGNOSIS

                PATHOLOGY

                • General Features

                  • Gross Pathologic & Surgical Features

                    • Microscopic Features

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              DIAGNOSTIC CHECKLIST

                              • Consider

                                • Image Interpretation Pearls

                                  Selected References

                                  1. Fretheim H et al: Fecal microbiota transplantation in systemic sclerosis: a double-blind, placebo-controlled randomized pilot trial. PLoS One. 15(5):e0232739, 2020
                                  2. Stupalkowska W et al: Spontaneous bowel perforation in the setting of colonic involvement with scleroderma. BMJ Case Rep. 13(1), 2020
                                  3. Sakkas LI et al: Intestinal involvement in systemic sclerosis: a clinical review. Dig Dis Sci. 63(4):834-44, 2018
                                  4. Singh G et al: Pneumatosis cystoides intestinalis (PCI) in a patient with undiagnosed systemic sclerosis. BMJ Case Rep, 2018
                                  5. Venkatasamy A et al: The stack of coins sign in scleroderma. Abdom Radiol (NY). 43(10):2878-9, 2018
                                  6. Xiang H et al: Hidebound sign: small bowel in systemic sclerosis. J Med Imaging Radiat Oncol. 62 Suppl 1:80, 2018
                                  7. Savarino E et al: Gastrointestinal involvement in systemic sclerosis. Presse Med. 43(10 Pt 2):e279-91, 2014
                                  8. Zapatier JA et al: Intestinal obstruction and pseudo-obstruction in patients with systemic sclerosis. Acta Gastroenterol Latinoam. 43(3):227-30, 2013
                                  9. Balbir-Gurman A et al: Pneumatosis cystoides intestinalis in scleroderma-related conditions. Intern Med J. 42(3):323-9, 2012
                                  10. Ohkubo H et al: An epidemiologic survey of chronic intestinal pseudo-obstruction and evaluation of the newly proposed diagnostic criteria. Digestion. 86(1):12-9, 2012
                                  11. Forbes A et al: Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology (Oxford). 48 Suppl 3:iii36-9, 2009
                                  12. Koh CE et al: The internal anal sphincter in systemic sclerosis. Dis Colon Rectum. 52(2):315-8, 2009
                                  13. Domsic R et al: Gastrointestinal manifestations of systemic sclerosis. Dig Dis Sci. 53(5):1163-74, 2008
                                  14. Parodi A et al: Small intestinal bacterial overgrowth in patients suffering from scleroderma: clinical effectiveness of its eradication. Am J Gastroenterol. 103(5):1257-62, 2008
                                  15. Gregersen H et al: A new method for evaluation of intestinal muscle contraction properties: studies in normal subjects and in patients with systemic sclerosis. Neurogastroenterol Motil. 19(1):11-9, 2007
                                  16. Kudoh K et al: Gastrojejunostomy and duodenojejunostomy for megaduodenum in systemic sclerosis sine scleroderma: report of a case. Dig Dis Sci. 52(9):2257-60, 2007
                                  17. Mayes MD: Scleroderma epidemiology. Rheum Dis Clin North Am. 29(2):239-54, 2003
                                  18. Neef B et al: Image of the month: "hide-bound" bowel sign in scleroderma. Gastroenterology. 124(5):1179, 1567, 2003
                                  19. Goldblatt F et al: Antibody-mediated gastrointestinal dysmotility in scleroderma. Gastroenterology. 123(4):1144-50, 2002
                                  20. Coggins CA et al: Wide-mouthed sacculations in the esophagus: a radiographic finding in scleroderma. AJR Am J Roentgenol. 176(4):953-4, 2001
                                  21. Weston S et al: Clinical and upper gastrointestinal motility features in systemic sclerosis and related disorders. Am J Gastroenterol. 93(7):1085-9, 1998
                                  22. Lock G et al: Gastrointestinal manifestations of progressive systemic sclerosis. Am J Gastroenterol. 92(5):763-71, 1997
                                  23. Ott DJ: Esophageal motility disorders. Semin Roentgenol. 29(4):321-31, 1994
                                  24. Levine MS et al: Update on esophageal radiology. AJR Am J Roentgenol. 155(5):933-41, 1990
                                  25. Rohrmann CA Jr et al: Radiologic and histologic differentiation of neuromuscular disorders of the gastrointestinal tract: visceral myopathies, visceral neuropathies, and progressive systemic sclerosis. AJR Am J Roentgenol. 143(5):933-41, 1984
                                  Related Anatomy
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                                  Related Differential Diagnoses
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                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Top Differential Diagnoses

                                        • Diagnostic Checklist

                                          TERMINOLOGY

                                          • Synonyms

                                            • Progressive systemic sclerosis

                                          • Definitions

                                            • Multisystem disorder of small vessels and connective tissue of unknown etiology

                                          IMAGING

                                          • General Features

                                            • Radiographic Findings

                                              DIFFERENTIAL DIAGNOSIS

                                                PATHOLOGY

                                                • General Features

                                                  • Gross Pathologic & Surgical Features

                                                    • Microscopic Features

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          • Natural History & Prognosis

                                                            • Treatment

                                                              DIAGNOSTIC CHECKLIST

                                                              • Consider

                                                                • Image Interpretation Pearls

                                                                  Selected References

                                                                  1. Fretheim H et al: Fecal microbiota transplantation in systemic sclerosis: a double-blind, placebo-controlled randomized pilot trial. PLoS One. 15(5):e0232739, 2020
                                                                  2. Stupalkowska W et al: Spontaneous bowel perforation in the setting of colonic involvement with scleroderma. BMJ Case Rep. 13(1), 2020
                                                                  3. Sakkas LI et al: Intestinal involvement in systemic sclerosis: a clinical review. Dig Dis Sci. 63(4):834-44, 2018
                                                                  4. Singh G et al: Pneumatosis cystoides intestinalis (PCI) in a patient with undiagnosed systemic sclerosis. BMJ Case Rep, 2018
                                                                  5. Venkatasamy A et al: The stack of coins sign in scleroderma. Abdom Radiol (NY). 43(10):2878-9, 2018
                                                                  6. Xiang H et al: Hidebound sign: small bowel in systemic sclerosis. J Med Imaging Radiat Oncol. 62 Suppl 1:80, 2018
                                                                  7. Savarino E et al: Gastrointestinal involvement in systemic sclerosis. Presse Med. 43(10 Pt 2):e279-91, 2014
                                                                  8. Zapatier JA et al: Intestinal obstruction and pseudo-obstruction in patients with systemic sclerosis. Acta Gastroenterol Latinoam. 43(3):227-30, 2013
                                                                  9. Balbir-Gurman A et al: Pneumatosis cystoides intestinalis in scleroderma-related conditions. Intern Med J. 42(3):323-9, 2012
                                                                  10. Ohkubo H et al: An epidemiologic survey of chronic intestinal pseudo-obstruction and evaluation of the newly proposed diagnostic criteria. Digestion. 86(1):12-9, 2012
                                                                  11. Forbes A et al: Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology (Oxford). 48 Suppl 3:iii36-9, 2009
                                                                  12. Koh CE et al: The internal anal sphincter in systemic sclerosis. Dis Colon Rectum. 52(2):315-8, 2009
                                                                  13. Domsic R et al: Gastrointestinal manifestations of systemic sclerosis. Dig Dis Sci. 53(5):1163-74, 2008
                                                                  14. Parodi A et al: Small intestinal bacterial overgrowth in patients suffering from scleroderma: clinical effectiveness of its eradication. Am J Gastroenterol. 103(5):1257-62, 2008
                                                                  15. Gregersen H et al: A new method for evaluation of intestinal muscle contraction properties: studies in normal subjects and in patients with systemic sclerosis. Neurogastroenterol Motil. 19(1):11-9, 2007
                                                                  16. Kudoh K et al: Gastrojejunostomy and duodenojejunostomy for megaduodenum in systemic sclerosis sine scleroderma: report of a case. Dig Dis Sci. 52(9):2257-60, 2007
                                                                  17. Mayes MD: Scleroderma epidemiology. Rheum Dis Clin North Am. 29(2):239-54, 2003
                                                                  18. Neef B et al: Image of the month: "hide-bound" bowel sign in scleroderma. Gastroenterology. 124(5):1179, 1567, 2003
                                                                  19. Goldblatt F et al: Antibody-mediated gastrointestinal dysmotility in scleroderma. Gastroenterology. 123(4):1144-50, 2002
                                                                  20. Coggins CA et al: Wide-mouthed sacculations in the esophagus: a radiographic finding in scleroderma. AJR Am J Roentgenol. 176(4):953-4, 2001
                                                                  21. Weston S et al: Clinical and upper gastrointestinal motility features in systemic sclerosis and related disorders. Am J Gastroenterol. 93(7):1085-9, 1998
                                                                  22. Lock G et al: Gastrointestinal manifestations of progressive systemic sclerosis. Am J Gastroenterol. 92(5):763-71, 1997
                                                                  23. Ott DJ: Esophageal motility disorders. Semin Roentgenol. 29(4):321-31, 1994
                                                                  24. Levine MS et al: Update on esophageal radiology. AJR Am J Roentgenol. 155(5):933-41, 1990
                                                                  25. Rohrmann CA Jr et al: Radiologic and histologic differentiation of neuromuscular disorders of the gastrointestinal tract: visceral myopathies, visceral neuropathies, and progressive systemic sclerosis. AJR Am J Roentgenol. 143(5):933-41, 1984