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Scleroderma (Systemic Sclerosis) and the GI Tract
Scleroderma commonly affects the gastrointestinal tract in 90% of patients, causing problems through fibrosis and impaired motility. Common issues include acid reflux, delayed stomach emptying, bacterial overgrowth from small intestine immobility, and constipation from slowed colonic transit. Diagnosis involves tests to evaluate motility and function like endoscopy, pH testing, scintigraphy and breath tests. Treatment aims to control symptoms through dietary changes, acid suppression, prokinetics and antibiotics, though current options cannot stop progression of gastrointestinal fibrosis.
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Scleroderma (Systemic Sclerosis) and the GI Tract
- 1. ©2003 RUSH UniversityMedical Center Scleroderma (Systemic Sclerosis) and the GI Tract Michael D Brown MD, MACM, FACP, FACG, AGAF Professor of Medicine Section of Digestive Diseases
- 2. ©2003 RUSH UniversityMedical Center SSc • Prevalence in the US is 240 per million • Women > Men • Ages 35-65 • Cause ?
- 3. ©2003 RUSH UniversityMedical Center
- 4. GI Tract Lining ©2003RUSH University Medical Center GI Tract Anatomy
- 5. GI Tract Function ©2003 RUSHUniversity Medical Center
- 6. SSC Almost alwaysaffects the GI tract… • Upper or lower GI tract involved in 90% of SSc patients. • Both diffuse and limited cutaneous forms • Affects ANY part of GI tract – From esophagus to anus • Slow or rapidly progressive ©2003 RUSH University Medical Center Old or Young
- 7. Grading the GItract in SSc ©2003 RUSH University Medical Center Grade 0 Blood vessel injury Grade 1 Nerve injury Grade 3 Muscular injury Grade 4 Scarring/Fibrosis
- 8. SSc in SpecificGI Organs ©2003 RUSH University Medical Center
- 9. Normal Swallowing ofFood ©2003 RUSH University Medical Center
- 10. Esophageal disorders ©2003 RUSHUniversity Medical Center • Esophageal motility abnormalities are the most common GI problem in SSc. – Low valve pressure between the stomach and esophagus – Low or no motility in the esophagus – Impaired coordination of contractions and the valve • Affects the bottom 2/3rds of the esophagus • 50-80% of patients get severe acid reflux • Can lead to strictures, Barrett’s esophagus, stenosis
- 11. GERD • Acid gets inappropriately intothe esophagus ©2003 RUSH University Medical Center
- 12. It’s not justheartburn… • Cough • Sore throat • Hoarseness • Chest pain • Burning tongue • Excess belching ©2003 RUSH University Medical Center
- 13. Stomach Disorders • Stomachproblems in 50% of SSc patients – Heartburn, nausea, vomiting, bloating, regurgitation, abdominal pain and fullness, early satiety. – Weight loss, fatigue, weakness • Impaired accommodation of stomach to food intake • Poor stomach emptying due to poor motility • Abnormal electrical conduction in the stomach ©2003 RUSH University Medical Center
- 14. Small Intestine • Slowintestinal transit in 40-88% of SSc patients • 65% have no symptoms • Complications of S..L..O..W motility: – Malabsorption – Bacterial Overgrowth – Pseudoobstruction – Pneumatosis Cystoides intestinalis • (PCI) ©2003 RUSH University Medical Center
- 15. Bacterial Overgrowth • SIBOis secondary to slow intestinal motility • Bacteria cleared by peristalsis (motility) from the lower small intestine instead colonize it – Block nutrient absorption – Damage the bowel wall – Cause B12 deficiency • Diarrhea, weight loss, bloating, abdominal pain ©2003 RUSH University Medical Center
- 16. SIBO • Normal andAbnormal Bacterial Distribution ©2003 RUSH University Medical Center
- 17. Intestinal Dysmotility • Motilitymay become so poor as to act as a obstruction to gut flow – i.e. “pseudoobstruction” • PCI=air in gut wall due to loss of wall compliance and scaring. ©2003 RUSH University Medical Center
- 18. Colon • Colonic involvementin 20-50% of SSc patients • Loss of colonic contractions and motility • Colonic pseudoobstructions ©2003 RUSH University Medical Center
- 19. Anorectum • Anorectal dysfunctionin 50-70% of SSc patients • Scarring of the anal sphincter – Outlet obstruction constipation – Fecal impaction – Rectal prolapse • Constipation • Urgency • Incontinence • Rectal fullness ©2003 RUSH University Medical Center Anus Rectum
- 20. Liver • Primary biliary cirrhosis •Autoimmune damage of small bile ducts in the liver • Seen in 2-22% of SSc patients • Slower progression to end stage liver disease in SSc patients ©2003 RUSH University Medical Center
- 21. Diagnostic GI Tests Usedin SSc Patients ©2003 RUSH University Medical Center
- 22. Lab Testing inSSc • Micronutrients and Vitamin levels to follow closely (every 3-6 months) – Vitamin C (particularly with skin involvement) – Selenium – Vitamin D – Pyridoxine (Vit B6) – Zinc – Folate ©2003 RUSH University Medical Center
- 23. GI Testing inSSc • Endoscopy – Used to examine GI anatomy and directly sample the GI tract – NOT useful in evaluating motility or function of the gut ©2003 RUSH University Medical Center
- 24. A view ofthe normal upper GI tract ©2003 RUSH University Medical Center
- 25. GI Testing inSSc • Motility Testing – Assesses esophageal contractions and lower esophageal sphincter function – Uses a pressure catheter inserted into the nose ©2003 RUSH University Medical Center
- 26. GI Testing inSSc • Bravo pH Testing – A measurement of acid exposure in the esophagus using a wireless radio probe – A measure of acid reflux – Can measure acid exposure over 24-96 hours ©2003 RUSH University Medical Center
- 27. GI Tract Testingin SSc • Barium Studies – Barium is swallowed or inserted via catheter or enema into the GI tract to obtain and image of the anatomy – Can be used to evaluate swallowing – Fluoroscopy or CT scans; both use contrast to image GI tract – ERCP ©2003 RUSH University Medical Center
- 28. GI Tract Testing:Hydrogen Breath Test • Used to assess for small intestinal bacterial overgrowth • Patient consumes a carbohydrate hydrogen source • Tests measure bacterial release of hydrogen gas in breath from undigested carbohydrate • The “Birthday Test” ©2003 RUSH University Medical Center
- 29. GI Tract Testing:Endoscopic Ultrasound • Allows a detailed view of the wall & interior of various GI organs – Esophagus – Stomach – Pancreas – Gallbladder – Rectum ©2003 RUSH University Medical Center
- 30. GI Tract Testing •Electrogastrogram – Used to assess stomach motility and contractions – Limited clinical use – Research tool ©2003 RUSH University Medical Center
- 31. GI Tract Testing •Scintography – A radioactive marker is injected or swallowed to measure GI motility and its effectiveness in moving the radio marker along the GI tract – Most commonly used to measure stomach emptying – Also used to esophageal emptying, reflux and colonic transit ©2003 RUSH University Medical Center
- 32. GI Tract Testing:Capsule Telemetry • SmartPill™ – Measures gut transit • Gastric, small bowel and colon – Done over 5 days • Video Capsule endoscopy – Allows a complete view of the small intestine – Finds small intestinal lining abnormalities – Done over 8 hours ©2003 RUSH University Medical Center
- 33. Treatment Options ©2003 RUSHUniversity Medical Center
- 34. Esophageal Treatments • Lifestylemodification: Reduces acid exposure in the esophagus – Sleep at 30°, no eating 3 hours before bedtime, avoid chocolate, mint, coffee, stop smoking, sleep on left side • Acid suppression: Stops acid production – Proton pump inhibitors; H2 Blockers • Zantac™, Nexium™, Prilosec™, Aciphex™, Dexilant™ • Prokinetic agents: Helps esophagus empty – Metoclopramide, domperidone • Reglan™ ©2003 RUSH University Medical Center
- 35. Novel esophageal treatments •Endoscopic correction of GERD – Injection, plication, suture, polymer injection • New medications to reduce sphincter relaxations – GABA B-receptor – Cannabinoid receptor 1 – CCK 1 receptor antagonists • Transcutaneous Nerve Stimulation (TENS) • Acupuncture ©2003 RUSH University Medical Center
- 36. Laparoscopic Anti-reflux Procedures • LARP:Surgical procedures to stop reflux by using the stomach to make an acid barrier. ©2003 RUSH University Medical Center
- 37. Stomach treatments • Prokinetics:Helps stomach empty, reduces nausea – Metoclopramide, domperidone • Antiemetics: Reduces nausea and vomiting – Compazine™, Tigan™, Zofran™ • Botulinum toxin injection into pylorus – Relaxes the valve that blocks stomach emptying • Gastric pacing: electrical stimulation to reduce symptoms • Gastrostomy-tube/Jejunostomy-tube – Allows for stomach suction and feeding below the stomach. – A last resort ©2003 RUSH University Medical Center
- 38. Intestinal Treatments • Difficultand aimed at control of symptoms – Diet: Low residue (fiber) diet, increase fluids, multiple small meals, liquid supplements • Intravenous nutrition (total parenteral nutrition) is required in some. – Antibiotics • Rotating antibiotics to clear bacterial overgrowth (Xifaxin™, Augmentin™, Doxycycline (careful with this one!) – Prokinetics • Metocopramide & domperidone occasionally effective • Erythromycin ineffective • Pyridostigmine 30mg daily; a new option – Octreotide • Reduces symptoms, can help the skin • …but can delay stomach emptying ©2003 RUSH University Medical Center
- 39. Low Fiber Foods ©2003RUSH University Medical Center Enriched white bread White rice Plain pasta, noodles or macaroni Cereals with no more than 1 gram of dietary fiber per serving Most canned or cooked fruits without skins, seeds or membranes Raw fruit without skin or membranes Fruit and vegetable juice with little or no pulp Canned or well-cooked vegetables without seeds, hulls or skins, such as carrots, string beans and peppers Tender meat, poultry and fish Eggs Smooth (creamy) peanut butter — up to 2 tablespoons a day Milk Yogurt or cheese without seeds or nuts Fats, oils and dressings without seeds Desserts with no seeds or nuts
- 40. Diet: FODMAP • FermentableOligosaccharides Disaccharides Monosaccharides and Polyols. – Fructose – Lactose – Fructans – Galactan – Polyols • Poorly absorbed short chain carbohydrates • Since 1970…artificial sweeteners = 22% increase in % of our total daily calorie intake
- 41. Physiology of FODMAPS •Poorly absorbed • Rapidly transported to colon • Highly fermented by gut microbiome • Results in: – Gas production – Bloating – Diarrhea – Abdominal pain – Cramping – Visceral hypersensitivity
- 42. Practical approach toa modified FODMAP diet • Use a Dietician! – Self directed FODMAP diets have not been shown to be effective • Define qualitatively and quantitatively the patients typical eating habits and lifestyle. • Explain the scientific basis for the FODMAP malabsorption and and subsequent fermentation to the patient • Provide specific dietary instructions • Discuss techniques for situations where food preparation cannot be controlled (restaurants, school, camp, friend’s home)
- 43. Intestinal Treatments • Surgery –Subtotal colectomy (removal of the colon) with colostomy for severe colonic inertia or slow transit – Resection of small bowel affected by severe pneumatosis cystoides intestinalis • Intestinal and colonic pacing – Animal studies only • Accupuncture/accupressure – Electroacupuncture @ GI acupoints – Animal studies only ©2003 RUSH University Medical Center
- 44. Anorectal Treatments • Stoolbulking agents (soluble fiber/bran) • Antidiarrheal drugs – Immodium™, Lomotil™ • Tricyclic antidepressants – Amitriptyline, desipramine • Surgery – Injectable silicone based bio-material – Implantable sacral nerve stimulator – Rectal prolapse repair • Accupuncture – Only studied in children w/o SSc ©2003 RUSH University Medical Center
- 45. Summary • The GItract is uniformly affected in patients with SSc of any sub-type • The motility of the GI tract is dysfunctional – …so the GI tract moves slowly – …and sphincters (valves) are weak • Diagnosis is fairly easy • Treatment is aimed at controlling symptoms • There are currently no treatments yet that will halt the progression of the fibrosis or scarring in the GI tract… ©2003 RUSH University Medical Center