1. Gastrointestinal features
Scleroderma
Gastrointestinal features
Iraj Salehi-Abari MD.,
Internist
Rheumatologist

2. Epidemiology: Annual incidence in USA: 1-2 / 100,000
Salehi I.
Epidemiology:
Annual incidence in USA: 1-2 / 100,000
Peak onset age: y/o
100 RA, 10 SLE, 1 Scl
F/M ratio: 3/1
Black > other race
Rare in children
Rare in men < 30 y/o
Scleroderma

3. General manifestations:
Salehi I.
General manifestations:
Most frequent:
Fatigue, Stiff joints
Loss of strength, Pain
Sleep difficulties
Skin discoloration
Less frequent:
Breathlessness, depression, sore eyes
Upset stomach, nausea, weight loss
Scleroderma

4. Organ involvement: Gastrointestinal Pulmonary Renal Cardiac
Salehi I.
Organ involvement:
Gastrointestinal
Pulmonary
Renal
Cardiac
Musculoskeletal
Ophthalmic
Others
Scleroderma

5. Gastrointestinal involvement:
Salehi I.
Gastrointestinal involvement:
In 90% of cases
Any part of GI
About 50% symptomatic
Esophageal: most common
Pathology:
. Smooth muscle atrophy
. Gut wall fibrosis
Scleroderma

6. Oral involvement: Micro-ostia (fish mouth)
Salehi I.
Oral involvement:
Micro-ostia (fish mouth)
Rigidity of tongue with short frenulum
Rigidity & tenderness of oral mucosa
Malalignment of teeth
Oropharyngeal dysphagia: 25%
Oropharyngeal incoordination: 25%
Sjogren’s syndrome
Impaired mastication & deglutition
Scleroderma

7. Esophageal involvement (90%):
Salehi I.
Esophageal involvement (90%):
Lower two-thirds of Esophagus:
Reduced or No peristalsis
Dilated lumen
Decreased LES tone
GERD: pyrosis
Dysmotility: dysphagia
Hiatal hernia
Scleroderma

8. Esophageal involvement (90%):
Salehi I.
Esophageal involvement (90%):
In past: Manometry, Barium swallow
Nowadays: Esophagoscopy if:
Complications are suspected
Persistent symptoms after PPI therapy
Esophagoscopy:
Reflux esophagitis, candidiasis
Barrett’s esophagus, LE stricture
Scleroderma

9. Gastric involvement: Gastric emptying: delayed Mucosal telangiectasia:
Salehi I.
Gastric involvement:
Gastric emptying: delayed
Mucosal telangiectasia:
Watermelon stomach
Upper GI bleeding
Gastric antral venous ectasia (GAVE):
Unexplained IDA
Gastroparesis: rare
Scleroderma

10. Salehi I.
Watermelon stomach
Scleroderma

11. Small bowel involvement:
Salehi I.
Small bowel involvement:
Reduced peristalsis
Abdominal distension
Bacterial overgrowth
Intermittent diarrhea-constipation
Malabsorption
Pseudo-obstruction & Perforation
Pneumatosis cystoides intestinalis
Volvulus
Pseudo-obstruction
Pneumatosis intestinalis
Rectal prolaps, incontinence
Scleroderma

12. Colonic disease: Anorectum: most common Wide-mouthed diverticuli
Salehi I.
Colonic disease:
Anorectum: most common
Fecal incontinence
Rectal prolapse
Gastrocolic reflex: absent
Wide-mouthed diverticuli
Constipation
Colonic perforation
Colonic infarction
Scleroderma