Esophagus
Nonneoplastic disorders
Scleroderma (systemic sclerosis)

Author: Elliot Weisenberg, M.D. (see Authors page)

Revised: 17 January 2018, last major update December 2012

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

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

Cite this page: Weisenberg, E. Scleroderma (systemic sclerosis). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophagusscleroderma.html. Accessed September 22nd, 2018.
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
Treatment
  • 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

Images hosted on other servers:

Distal and proximal oesophagus

Severe atrophy of circular muscle

Myenteric plexus

Interstitial cells of Cajal


Fibrosis of muscularis propria

Trichrome stain

Barrett esophagus

Electron microscopy images

Images hosted on other servers:

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

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

Differential diagnosis