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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
Fretheim H et al: Fecal microbiota transplantation in systemic sclerosis: a double-blind, placebo-controlled randomized pilot trial. PLoS One. 15(5):e0232739, 2020
Stupalkowska W et al: Spontaneous bowel perforation in the setting of colonic involvement with scleroderma. BMJ Case Rep. 13(1), 2020
Sakkas LI et al: Intestinal involvement in systemic sclerosis: a clinical review. Dig Dis Sci. 63(4):834-44, 2018
Singh G et al: Pneumatosis cystoides intestinalis (PCI) in a patient with undiagnosed systemic sclerosis. BMJ Case Rep, 2018
Venkatasamy A et al: The stack of coins sign in scleroderma. Abdom Radiol (NY). 43(10):2878-9, 2018
Xiang H et al: Hidebound sign: small bowel in systemic sclerosis. J Med Imaging Radiat Oncol. 62 Suppl 1:80, 2018
Savarino E et al: Gastrointestinal involvement in systemic sclerosis. Presse Med. 43(10 Pt 2):e279-91, 2014
Zapatier JA et al: Intestinal obstruction and pseudo-obstruction in patients with systemic sclerosis. Acta Gastroenterol Latinoam. 43(3):227-30, 2013
Balbir-Gurman A et al: Pneumatosis cystoides intestinalis in scleroderma-related conditions. Intern Med J. 42(3):323-9, 2012
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
Forbes A et al: Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology (Oxford). 48 Suppl 3:iii36-9, 2009
Koh CE et al: The internal anal sphincter in systemic sclerosis. Dis Colon Rectum. 52(2):315-8, 2009
Domsic R et al: Gastrointestinal manifestations of systemic sclerosis. Dig Dis Sci. 53(5):1163-74, 2008
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
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
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
Neef B et al: Image of the month: "hide-bound" bowel sign in scleroderma. Gastroenterology. 124(5):1179, 1567, 2003
Goldblatt F et al: Antibody-mediated gastrointestinal dysmotility in scleroderma. Gastroenterology. 123(4):1144-50, 2002
Coggins CA et al: Wide-mouthed sacculations in the esophagus: a radiographic finding in scleroderma. AJR Am J Roentgenol. 176(4):953-4, 2001
Weston S et al: Clinical and upper gastrointestinal motility features in systemic sclerosis and related disorders. Am J Gastroenterol. 93(7):1085-9, 1998
Lock G et al: Gastrointestinal manifestations of progressive systemic sclerosis. Am J Gastroenterol. 92(5):763-71, 1997
Ott DJ: Esophageal motility disorders. Semin Roentgenol. 29(4):321-31, 1994
Levine MS et al: Update on esophageal radiology. AJR Am J Roentgenol. 155(5):933-41, 1990
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
Fretheim H et al: Fecal microbiota transplantation in systemic sclerosis: a double-blind, placebo-controlled randomized pilot trial. PLoS One. 15(5):e0232739, 2020
Stupalkowska W et al: Spontaneous bowel perforation in the setting of colonic involvement with scleroderma. BMJ Case Rep. 13(1), 2020
Sakkas LI et al: Intestinal involvement in systemic sclerosis: a clinical review. Dig Dis Sci. 63(4):834-44, 2018
Singh G et al: Pneumatosis cystoides intestinalis (PCI) in a patient with undiagnosed systemic sclerosis. BMJ Case Rep, 2018
Venkatasamy A et al: The stack of coins sign in scleroderma. Abdom Radiol (NY). 43(10):2878-9, 2018
Xiang H et al: Hidebound sign: small bowel in systemic sclerosis. J Med Imaging Radiat Oncol. 62 Suppl 1:80, 2018
Savarino E et al: Gastrointestinal involvement in systemic sclerosis. Presse Med. 43(10 Pt 2):e279-91, 2014
Zapatier JA et al: Intestinal obstruction and pseudo-obstruction in patients with systemic sclerosis. Acta Gastroenterol Latinoam. 43(3):227-30, 2013
Balbir-Gurman A et al: Pneumatosis cystoides intestinalis in scleroderma-related conditions. Intern Med J. 42(3):323-9, 2012
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
Forbes A et al: Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology (Oxford). 48 Suppl 3:iii36-9, 2009
Koh CE et al: The internal anal sphincter in systemic sclerosis. Dis Colon Rectum. 52(2):315-8, 2009
Domsic R et al: Gastrointestinal manifestations of systemic sclerosis. Dig Dis Sci. 53(5):1163-74, 2008
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
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
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
Neef B et al: Image of the month: "hide-bound" bowel sign in scleroderma. Gastroenterology. 124(5):1179, 1567, 2003
Goldblatt F et al: Antibody-mediated gastrointestinal dysmotility in scleroderma. Gastroenterology. 123(4):1144-50, 2002
Coggins CA et al: Wide-mouthed sacculations in the esophagus: a radiographic finding in scleroderma. AJR Am J Roentgenol. 176(4):953-4, 2001
Weston S et al: Clinical and upper gastrointestinal motility features in systemic sclerosis and related disorders. Am J Gastroenterol. 93(7):1085-9, 1998
Lock G et al: Gastrointestinal manifestations of progressive systemic sclerosis. Am J Gastroenterol. 92(5):763-71, 1997
Ott DJ: Esophageal motility disorders. Semin Roentgenol. 29(4):321-31, 1994
Levine MS et al: Update on esophageal radiology. AJR Am J Roentgenol. 155(5):933-41, 1990
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
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