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Esophageal Scleroderma
Atif Zaheer, MD; Michael P. Federle, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        TERMINOLOGY

        • Synonyms

          • Progressive systemic sclerosis
        • Definitions

          • Multisystem disorder of small vessels and connective tissue (collagen vascular disease)

        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. Chung JH et al: Imaging features of systemic sclerosis-associated interstitial lung disease. J Vis Exp. 160, 2020
                                2. Fraticelli P et al: Videofluorography swallow study in patients with systemic sclerosis: correlation with clinical and radiological features. Clin Exp Rheumatol. 37 Suppl 119(4):108-14, 2019
                                3. Lee MH et al: Clinical, imaging, and pathologic features of conditions with combined esophageal and cutaneous manifestations. Radiographics. 39(5):1411-34, 2019
                                4. McMahan ZH: Gastrointestinal involvement in systemic sclerosis: an update. Curr Opin Rheumatol. 31(6):561-8, 2019
                                5. Orlandi M et al: One year in review 2018: systemic sclerosis. Clin Exp Rheumatol. 36 Suppl 113(4):3-23, 2018
                                6. Denton CP et al: Systemic sclerosis. Lancet. 390(10103):1685-99, 2017
                                7. Emmanuel A: Current management of the gastrointestinal complications of systemic sclerosis. Nat Rev Gastroenterol Hepatol. 13(8):461-72, 2016
                                8. Simeón-Aznar CP et al: Systemic sclerosis sine scleroderma and limited cutaneous systemic sclerosis: similarities and differences. Clin Exp Rheumatol. 32(6 Suppl 86):S-33-40, 2014
                                9. Savarino E et al: Gastroesophageal reflux and pulmonary fibrosis in scleroderma: a study using pH-impedance monitoring. Am J Respir Crit Care Med. 179(5):408-13, 2009
                                10. Hinchcliff M et al: Systemic sclerosis/scleroderma: a treatable multisystem disease. Am Fam Physician. 78(8):961-8, 2008
                                11. Vonk MC et al: Oesophageal dilatation on high-resolution computed tomography scan of the lungs as a sign of scleroderma. Ann Rheum Dis. 67(9):1317-21, 2008
                                12. Sifrim D et al: Non-achalasic motor disorders of the oesophagus. Best Pract Res Clin Gastroenterol. 21(4):575-93, 2007
                                13. Ntoumazios SK et al: Esophageal involvement in scleroderma: gastroesophageal reflux, the common problem. Semin Arthritis Rheum. 36(3):173-81, 2006
                                14. Mayes MD: Scleroderma epidemiology. Rheum Dis Clin North Am. 29(2):239-54, 2003
                                15. Goldblatt F et al: Antibody-mediated gastrointestinal dysmotility in scleroderma. Gastroenterology. 123(4):1144-50, 2002
                                16. Coggins CA et al: Wide-mouthed sacculations in the esophagus: a radiographic finding in scleroderma. AJR Am J Roentgenol. 176(4):953-4, 2001
                                17. Duchini A et al: Gastrointestinal hemorrhage in patients with systemic sclerosis and CREST syndrome. Am J Gastroenterol. 93(9):1453-6, 1998
                                18. Weston S et al: Clinical and upper gastrointestinal motility features in systemic sclerosis and related disorders. Am J Gastroenterol. 93(7):1085-9, 1998
                                19. Lock G et al: Gastrointestinal manifestations of progressive systemic sclerosis. Am J Gastroenterol. 92(5):763-71, 1997
                                20. Young MA et al: Gastrointestinal manifestations of scleroderma. Rheum Dis Clin North Am. 22(4):797-823, 1996
                                21. Kahan A et al: Gastrointestinal involvement in systemic sclerosis. Clin Dermatol. 12(2):259-65, 1994
                                22. Ott DJ: Esophageal motility disorders. Semin Roentgenol. 29(4):321-31, 1994
                                23. Sjogren RW: Gastrointestinal motility disorders in scleroderma. Arthritis Rheum. 37(9):1265-82, 1994
                                24. Levine MS et al: Update on esophageal radiology. AJR Am J Roentgenol. 155(5):933-41, 1990
                                25. Marshall JB et al: Gastrointestinal manifestations of mixed connective tissue disease. Gastroenterology. 98(5 Pt 1):1232-8, 1990
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Top Differential Diagnoses

                                      TERMINOLOGY

                                      • Synonyms

                                        • Progressive systemic sclerosis
                                      • Definitions

                                        • Multisystem disorder of small vessels and connective tissue (collagen vascular disease)

                                      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. Chung JH et al: Imaging features of systemic sclerosis-associated interstitial lung disease. J Vis Exp. 160, 2020
                                                              2. Fraticelli P et al: Videofluorography swallow study in patients with systemic sclerosis: correlation with clinical and radiological features. Clin Exp Rheumatol. 37 Suppl 119(4):108-14, 2019
                                                              3. Lee MH et al: Clinical, imaging, and pathologic features of conditions with combined esophageal and cutaneous manifestations. Radiographics. 39(5):1411-34, 2019
                                                              4. McMahan ZH: Gastrointestinal involvement in systemic sclerosis: an update. Curr Opin Rheumatol. 31(6):561-8, 2019
                                                              5. Orlandi M et al: One year in review 2018: systemic sclerosis. Clin Exp Rheumatol. 36 Suppl 113(4):3-23, 2018
                                                              6. Denton CP et al: Systemic sclerosis. Lancet. 390(10103):1685-99, 2017
                                                              7. Emmanuel A: Current management of the gastrointestinal complications of systemic sclerosis. Nat Rev Gastroenterol Hepatol. 13(8):461-72, 2016
                                                              8. Simeón-Aznar CP et al: Systemic sclerosis sine scleroderma and limited cutaneous systemic sclerosis: similarities and differences. Clin Exp Rheumatol. 32(6 Suppl 86):S-33-40, 2014
                                                              9. Savarino E et al: Gastroesophageal reflux and pulmonary fibrosis in scleroderma: a study using pH-impedance monitoring. Am J Respir Crit Care Med. 179(5):408-13, 2009
                                                              10. Hinchcliff M et al: Systemic sclerosis/scleroderma: a treatable multisystem disease. Am Fam Physician. 78(8):961-8, 2008
                                                              11. Vonk MC et al: Oesophageal dilatation on high-resolution computed tomography scan of the lungs as a sign of scleroderma. Ann Rheum Dis. 67(9):1317-21, 2008
                                                              12. Sifrim D et al: Non-achalasic motor disorders of the oesophagus. Best Pract Res Clin Gastroenterol. 21(4):575-93, 2007
                                                              13. Ntoumazios SK et al: Esophageal involvement in scleroderma: gastroesophageal reflux, the common problem. Semin Arthritis Rheum. 36(3):173-81, 2006
                                                              14. Mayes MD: Scleroderma epidemiology. Rheum Dis Clin North Am. 29(2):239-54, 2003
                                                              15. Goldblatt F et al: Antibody-mediated gastrointestinal dysmotility in scleroderma. Gastroenterology. 123(4):1144-50, 2002
                                                              16. Coggins CA et al: Wide-mouthed sacculations in the esophagus: a radiographic finding in scleroderma. AJR Am J Roentgenol. 176(4):953-4, 2001
                                                              17. Duchini A et al: Gastrointestinal hemorrhage in patients with systemic sclerosis and CREST syndrome. Am J Gastroenterol. 93(9):1453-6, 1998
                                                              18. Weston S et al: Clinical and upper gastrointestinal motility features in systemic sclerosis and related disorders. Am J Gastroenterol. 93(7):1085-9, 1998
                                                              19. Lock G et al: Gastrointestinal manifestations of progressive systemic sclerosis. Am J Gastroenterol. 92(5):763-71, 1997
                                                              20. Young MA et al: Gastrointestinal manifestations of scleroderma. Rheum Dis Clin North Am. 22(4):797-823, 1996
                                                              21. Kahan A et al: Gastrointestinal involvement in systemic sclerosis. Clin Dermatol. 12(2):259-65, 1994
                                                              22. Ott DJ: Esophageal motility disorders. Semin Roentgenol. 29(4):321-31, 1994
                                                              23. Sjogren RW: Gastrointestinal motility disorders in scleroderma. Arthritis Rheum. 37(9):1265-82, 1994
                                                              24. Levine MS et al: Update on esophageal radiology. AJR Am J Roentgenol. 155(5):933-41, 1990
                                                              25. Marshall JB et al: Gastrointestinal manifestations of mixed connective tissue disease. Gastroenterology. 98(5 Pt 1):1232-8, 1990