Gastrointestinal Manifestations of Systemic Sclerosis

Case Report

Figure 1 · Endoscopic findings

Panel A: Esophageal Motility Disorder With Dilation And Retained Secretions
(A) Esophageal motility disorder with dilation and retained secretions.
Panel B: Megaduodenum With Wide Separation Between Kerckring Folds
(B) Megaduodenum with wide separation between Kerckring folds.
Panel C: Mega Ileum With Mucosal Erythema
(C) Mega ileum with mucosal erythema.

Experienced teaching points

Clinical Pearls

01Systemic sclerosis commonly affects the gastrointestinal tract through collagen deposition and muscular atrophy.

02Endoscopic evaluation may reveal esophageal dysmotility with dilation, megaduodenum, and mega ileum.

03Dilated bowel segments can delay transit and predispose to secondary bacterial overgrowth.

04Recognizing these patterns is essential for comprehensive management of scleroderma-related GI disease.

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Final Diagnosis

Esophageal motility disorder, mega duodenum, and mega ileum associated with systemic sclerosis.

Clinical History

A 48-year-old woman with known systemic sclerosis was referred for malignancy screening. She reported dyspepsia and abdominal bloating. Her medications included mycophenolate mofetil. On clinical examination, her abdomen was slightly distended and tympanitic but non-tender. The indication for endoscopy was malignancy screening and evaluation of her gastrointestinal symptoms.

Endoscopic Findings

  1. Esophageal motility disorder and dilation with retained saliva.
  2. Markedly dilated duodenum with wide separation between Kerckring folds.
  3. Markedly dilated terminal ileum on colonoscopy.

Endoscopic Technique

Standard esophagogastroduodenoscopy and colonoscopy with terminal ileum intubation were performed for malignancy screening and symptom evaluation. No therapeutic intervention was required during this diagnostic examination.

Discussion

Mechanism. Systemic sclerosis, or scleroderma, is characterized by widespread collagen deposition, resulting in tissue and organ fibrosis. Most patients experience gastrointestinal manifestations, including esophageal dysmotility, gastroparesis, megaduodenum, small bowel diverticulosis, and chronic intestinal pseudo-obstruction. The gastrointestinal abnormalities are likely a consequence of muscular atrophy and collagen replacement of the outer longitudinal layer relative to the inner circular layer. Small intestinal involvement can lead to delayed transit, dilation of small bowel loops, and secondary bacterial overgrowth.

What the scope shows. In this patient, the endoscopic triad of esophageal dilation with retained secretions, megaduodenum, and mega ileum illustrates how scleroderma can remodel multiple GI segments at once.

Why it matters. Recognition matters because these findings explain common symptoms such as dyspepsia and bloating, and they raise the possibility of bacterial overgrowth when dilated loops impair clearance.

References

  1. Prakash R, Saxena A. Gastrointestinal manifestations of systemic sclerosis. Gastroenterol Res Pract. 2017;2017:7102157.
  2. Runchey S, Sharma A. Esophageal dysmotility in systemic sclerosis: A review. J Clin Gastroenterol. 2021;55(8):666-673.
  3. Abu-Amara M, et al. Small bowel involvement in systemic sclerosis: Clinical and endoscopic features. World J Gastroenterol. 2011;17(30):3594-3599.
  4. Hokama A. Megaduodenum in systemic sclerosis. Rheumatology. 2022;61:e194.

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