Esophageal Scleroderma

Published on 13/07/2015 by admin

Filed under Radiology

Last modified 13/07/2015

Print this page

rate 1 star rate 2 star rate 3 star rate 4 star rate 5 star
Your rating: none, Average: 0 (0 votes)

This article have been viewed 2095 times

 GI tract, lungs, heart, kidneys, and nervous system

• GI: 3rd most common manifestation after skin changes and Raynaud phenomenon

image Seen in up to 90% of patients
image Most common sites

– Esophagus > duodenum > anorectal > small bowel > colon
• Interstitial pulmonary fibrosis, often severe
• Scleroderma is classified into 2 types

image Diffuse scleroderma and CREST syndrome
• Diffuse scleroderma

image Cutaneous and visceral involvement 

– Often severe
• CREST syndrome: Minimal cutaneous and late visceral

image C: Calcinosis of skin
image R: Raynaud phenomenon
image E: Esophageal dysmotility
image S: Sclerodactyly (involvement of fingers)
image T: Telangiectasia
• Esophagography

image Atony or aperistalsis: Lower 2/3 (smooth muscle)
image Mild to moderate dilatation of esophagus
image Patulous lower esophageal sphincter (early)
image Ulcers, fusiform peptic stricture (later)
image Gastroesophageal reflux (70% of patients)
image 40% develop Barrett esophagus

TOP DIFFERENTIAL DIAGNOSES

• Esophageal achalasia
• Reflux esophagitis (with stricture)
• Esophageal carcinoma
• Iatrogenic
image
(Left) Upright film from an esophagram in a 29-year-old woman with dysphagia and shortness of breath shows a dilated, atonic esophagus image with a distal esophageal stricture image. Esophageal peristalsis was completely absent.

image
(Right) Chest CT in the same patient shows interstitial fibrosis and a massive dilated esophagus image, all findings due to scleroderma.
image
(Left) Film from an esophagram in a young woman with dysphagia shows a dilated esophagus with a persistent air-fluid level, indicating delayed emptying. There is stricture of the distal esophagus image.

image
(Right) Subsequent film in the same patient shows a dilated duodenum with functional narrowing of its 3rd portion. The duodenum is often dilated and atonic in patients with scleroderma.

TERMINOLOGY

Synonyms

• Progressive systemic sclerosis

Definitions

• Multisystem disorder of small vessels and connective tissue (collagen vascular disease) of unknown etiology

IMAGING

General Features

• Best diagnostic clue

image Dilated atonic esophagus with distal stricture (late findings)
• Other general features

image Multisystemic disorder with immunologic and inflammatory changes
image Characterized by atrophy, fibrosis, and sclerosis of skin, vessels, and organs
image Involves skin, synovium, and parenchyma of multiple organs

– Gastrointestinal tract, lungs, heart, kidneys, and nervous system
image Gastrointestinal (GI) tract scleroderma

– 3rd most common manifestation after skin changes and Raynaud phenomenon
– Seen in up to 90% of patients
– Most common sites: Esophagus > duodenum > anus/rectum > small bowel > colon
– Most frequent cause of chronic intestinal pseudo-obstruction
image Scleroderma is classified into 2 types

– Diffuse scleroderma
– CREST syndrome (more benign course)
image Diffuse scleroderma: Cutaneous and visceral involvement

– Interstitial pulmonary fibrosis, often severe
– Organ failure more likely
– Associated with antitopoisomerase 1 antibody (anti-Scl 70)
image CREST syndrome: Minimal cutaneous and late visceral involvement

– C: Calcinosis of skin
– R: Raynaud phenomenon
– E: Esophageal dysmotility
– S: Sclerodactyly (involvement of fingers)
– T: Telangiectasia
– Associated with anticentromere antibodies

Radiographic Findings

• Fluoroscopic-guided esophagography

image Normal peristalsis above aortic arch (striated muscle)
image Atony or aperistalsis: Lower 2/3 (smooth muscle)
image Mild to moderate dilatation of esophagus
image Patulous lower esophageal sphincter (LES)

– Early finding of scleroderma
image Erosions, superficial ulcers, fusiform peptic stricture

– Due to reflux esophagitis
image Gastroesophageal reflux (70% cases)

– 40% develop Barrett esophagus
image Hiatal hernia
• 

Buy Membership for Radiology Category to continue reading. Learn more here