Gastrointestinal features Scleroderma Gastrointestinal features Iraj Salehi-Abari MD., Internist Rheumatologist salehiabari@sina.tums.ac.ir

Epidemiology: Annual incidence in USA: 1-2 / 100,000 Salehi I. Epidemiology: Annual incidence in USA: 1-2 / 100,000 Peak onset age: 30-50 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

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

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

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

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

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

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

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

Salehi I. Watermelon stomach Scleroderma

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

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