Other nonneoplastic

Scleroderma (systemic sclerosis)

Last author update: 1 December 2012
Last staff update: 17 December 2020

Copyright: 2003-2023,, Inc.

PubMed Search: Scleroderma[TI] OR systemic sclerosis[TI] esophagus[TI]

Elliot Weisenberg, M.D.
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Cite this page: Weisenberg E. Scleroderma (systemic sclerosis). website. Accessed November 29th, 2023.
Definition / general
  • Characterized by fibrosis, inflammation, increased collagen and vasculitis
  • Can be part of CREST syndrome (Calcinosis, Raynaud phenomenon, Esophageal involvement, Sclerodactyly, Telangiectasia)
  • Involves esophagus in 75%+ patients, usually distal 2/3, with aperistalsis and reduced tone of lower esophageal sphincter
  • Commonly causes reflux esophagitis (Semin Arthritis Rheum 2006;36:173), strictures, Candida esophagitis, Barrett esophagus (J Clin Gastroenterol 2006;40:769 but similar prevalence of Barrett as other reflux esophagitis patients, Arthritis Rheum 2005;52:2882)
Case reports
  • No treatment for underlying disease; proton pump inhibitors for reflux esophagitis, dilation for strictures
Microscopic (histologic) description
  • Resection specimens: atrophy and replacement fibrosis of inner circular layer of muscularis propria and resulting stenosis; longitudinal layer is usually involved; also submucosal fibrosis, mild inflammation, intimal proliferation of arterioles (Gut 2006;55:1697)
  • Biopsies: ulcers or erosions resembling reflux esophagitis, Candida or Barrett
Microscopic (histologic) images

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Distal and proximal oesophagus

Severe atrophy of circular muscle

Myenteric plexus

Interstitial cells of Cajal

Barrett esophagus

Electron microscopy images

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Collagen between
degenerate muscle
fiber (left) and necrotic
muscle fiber (right)

Muscle fibers (M)
separated by collagenous
fibers with widened
intermyofibrillar space

Differential diagnosis
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Image 01 Image 02