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Year : 2017  |  Volume : 12  |  Issue : 2  |  Page : 118-119

Scleroderma esophagus: A case series

Department of Gastroenterology, Choithram Hospital and Research Centre, Indore, Madhya Pradesh; Department of Gastroenterology, Pushpavati Singhanaia Research Institute, New Delhi, India

Date of Web Publication26-May-2017

Correspondence Address:
Mayank Jain
297, Indrapuri, Near Bhanwarkuan, Indore - 452 017, Madhya Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-3698.202126

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How to cite this article:
Jain M, Baijal R. Scleroderma esophagus: A case series. Indian J Rheumatol 2017;12:118-9

How to cite this URL:
Jain M, Baijal R. Scleroderma esophagus: A case series. Indian J Rheumatol [serial online] 2017 [cited 2023 Feb 5];12:118-9. Available from:

Dear Editor,

Systemic sclerosis causes smooth muscle atrophy and fibrosis of the distal two-thirds of the esophagus. This leads to symptoms of dysphagia, heartburn, and regurgitation. Complications such as strictures and Barrett's esophagus are found in 17%–29% of patients and 0%–37% of patients, respectively.[1]

Due to the rarity of the disorder and limited access to high-end investigations, involvement of the esophagus in scleroderma is under reported from India. In this study, we report our experience of six cases of diffuse scleroderma that were referred for the evaluation of dysphagia between January 2014 and January 2016. This retrospective study data were collected from hospital records, and ethical committee approval was obtained. All were females with a mean age of 43.6 years (range 38–53 years) and mean duration of disease of 8.2 years (range 3–13 years). They were on multiple medications including immunosuppressants (mycophenolate mofetil-2, azathioprine-4), nifedipine (3), analgesics (6), and took antacids regularly for symptom relief. The presenting symptoms included dysphagia for solids (5/6), retrosternal pain (4/6), regurgitation (3/6), and weight loss (2/6). Upper gastrointestinal endoscopy showed severe reflux esophagitis in all cases (Los Angeles [LA] class C-4, Class D-2). Esophageal manometry [Figure 1] was performed in all cases, and the findings included hypotensive lower esophageal sphincter (6/6), normal integrated relaxation pressure, and >50% of ineffective swallows. The ineffective swallows mainly showed hypotensive or absent peristalsis in distal 2/3 of the esophagus (4/6) and nonspecific dysmotility with large breaks (1/6). After evaluation, all patients were counseled regarding lifestyle changes for reflux symptoms and given twice a day pantoprazole (80 mg/day) and prokinetics (itopride 50 mg three times a day). Five patients reported partial relief in symptoms, whereas one patient was lost to follow-up. Endoscopy was repeated in only one patient after 8 weeks of treatment and showed esophagitis LA class A. None of the other patients consented for a repeat endoscopy or manometry.
Figure 1: Representative image of changes noted in a case of scleroderma

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Esophageal involvement is frequent in systemic sclerosis. Careful examination of the patients reveals gastrointestinal abnormalities even in patients without symptoms.[2] The role of such testing remains doubtful in morphea due to conflicting reports from various centers.[3],[4] Esophageal motor disorders in systemic sclerosis are linked to the severity of reflux disease, and the presence of reflux symptoms acts as simple warning sign which should prompt an evaluation by manometry.[5] In a large study of 194 patients with scleroderma, esophageal symptoms were present in 118 cases (60.8%). Reflux esophagitis was found in 73 cases (37.6%); it was mild or moderate in 47 cases (24.2%) and severe or complicated in the remaining cases. Manometry revealed a lower esophageal sphincter incompetence and esophageal motor disorders, respectively, in 118 (60.8%) and 157 cases (80.9%).[5] This study is our limited experience with this uncommon disorder. We noted a favorable response to lifestyle changes and drug therapy. To conclude, esophageal involvement is more frequent in diffuse scleroderma. Early evaluation of all patients with endoscopy (with manometry if possible) may help accurately diagnose the condition and institute appropriate therapy.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Ebert EC. Esophageal disease in scleroderma. J Clin Gastroenterol 2006;40:769-75.  Back to cited text no. 1
Lahcene M, Oumnia N, Matougui N, Boudjella M, Tebaibia A, Touchene B. Esophageal dysmotility in scleroderma: A prospective study of 183 cases. Gastroenterol Clin Biol 2009;33:466-9.  Back to cited text no. 2
Arif T, Masood Q, Singh J, Hassan I. Assessment of esophageal involvement in systemic sclerosis and morphea (localized scleroderma) by clinical, endoscopic, manometric and pH metric features: A prospective comparative hospital based study. BMC Gastroenterol 2015;15:24.  Back to cited text no. 3
Rose S, Young MA, Reynolds JC. Gastrointestinal manifestations of scleroderma. Gastroenterol Clin North Am 1998;27:563-94.  Back to cited text no. 4
Lahcene M, Oumnia N, Matougui N, Boudjella M, Tebaibia A, Touchene B. Esophageal involvement in scleroderma: Clinical, endoscopic, and manometric features. ISRN Rheumatol 2011;2011:325826.  Back to cited text no. 5


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