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Scleroderma and the GI tract
- 1. ©2003 RUSH University Medical Center Scleroderma (Systemic Sclerosis or SSc) and the GI Tract: Update 2023 Michael D Brown MD, MACM, FACP, FACG, AGAF Professor of Medicine Section of Digestive Diseases & Nutrition Rush University Medical Center
- 2. ©2003 RUSH University Medical Center
- 3. GI Tract Function ©2003 RUSH University Medical Center
- 4. SSC Almost always affects 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
- 5. Grading the GI tract in SSc ©2003 RUSH University Medical Center Grade 0 Blood vessel injury Grade 1 Nerve injury Grade 3 Muscular injury Grade 4 Scarring/Fibrosis
- 6. Causes of SSc and Possible GI Tract Origin ©2003 RUSH University Medical Center
- 7. ©2003 RUSH University Medical Center
- 8. • The colon contains trillions of organisms • This organisms have a HUGE ipact on human health • Alterations particularly in early like can lead to: – Inflammatory disorders – Autoimmune disorders – Atopic (Allergy) disorders ©2003 RUSH University Medical Center
- 9. Dysbiosis • Dysbiosis: – Altered colonic microbiota composition • Has widespread effects in: – Lung – Skin – Areas of concern in SSc ©2003 RUSH University Medical Center
- 10. • Patients with SSc demonstrate dysbiosis – Increased levels of: • Bifidobacterium • Provotella • Lactobacillus • Fusobacterium • Gamma-Proteobacteria – Decreased levels of: • Clostridium • Faecalibacterium – Cause and effect relationship is uncertain however… ©2003 RUSH University Medical Center
- 11. • ….that said this unique ecologic change may perpetuate the abnormalities and clinical consequences seen in SSc. – Some enhance the inflammatory response increasing white blood cell activity in skin and lung – Some calm the immune system and decrease white blood cell activity – Some are invasive and not friendly (probiotics) – i.e pathobionts • So probiotics should help right? ©2003 RUSH University Medical Center
- 12. Let’s look at those names again… – Increased levels of: • Bifidobacterium • Provotella • Lactobacillus • Fusobacterium • Gamma-Proteobacteria – Decreased levels of: • Clostridium • Faecalibacterium ©2003 RUSH University Medical Center Aren’t those 2 in my probiotic tablet?!
- 13. • Yes they are… – Why there is no simple answer to fixing dysbiosis – BOTH antibiotics and probiotics can worsen things – Not enough research done yet to predict what probiotics are best if any – Maybe in the future fecal microbiotia transplant? ©2003 RUSH University Medical Center
- 14. • …So how did the microbiota get that way in the first place? • New animal research suggests early life antibiotic exposure. • A SINGLE exposure to antibiotics in infancy seemed to increase risk of adult dysbiosis. ©2003 RUSH University Medical Center
- 15. Other risk factors for GI SSc • Helicobacter pylori infections – Conflicting data • Smoking – Data is weak in SSc but not in many other GI disorders ©2003 RUSH University Medical Center
- 16. GI Symptoms in SSc ©2003 RUSH University Medical Center
- 17. 98.9% of SSc patients have GI complaints • Meteorism or bloating • Heartburn • Trouble swallowing • Constipation/Diarrhea • Malabsorption with weight loss • Nausea • Vomiting • Bleeding • Dry mouth ©2003 RUSH University Medical Center
- 18. Consequences of GI Injury • Malnutrition • Intestinal pseudoobstruction • Ogilve Syndrome • Depression ©2003 RUSH University Medical Center
- 19. SSc in Specific GI Organs ©2003 RUSH University Medical Center
- 20. • Oral cavity • Esophagus • Stomach • Small Intestine • Colon • Rectum/Anus • Liver/bile ducts ©2003 RUSH University Medical Center
- 21. Oral Cavity ©2003 RUSH University Medical Center
- 22. Normal Swallowing of Food ©2003 RUSH University Medical Center
- 23. Esophageal disorders ©2003 RUSH University 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 • 75-90% of patients get severe acid reflux • Can lead to strictures, Barrett’s esophagus, stenosis
- 24. GERD • Acid gets inappropriately into the esophagus ©2003 RUSH University Medical Center
- 25. It’s not just heartburn… • Cough • Sore throat • Hoarseness • Chest pain • Burning tongue • Excess belching ©2003 RUSH University Medical Center
- 26. Stomach Disorders • Stomach problems 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 (gastroparesis) • Abnormal electrical conduction in the stomach • Blood vessel abnormalities (GAVE) ©2003 RUSH University Medical Center
- 27. G.A.V.E. • Gastric Antral Vascular Ectasia ©2003 RUSH University Medical Center
- 28. Small Intestine • Slow intestinal 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) – Jejunal diverticula ©2003 RUSH University Medical Center
- 29. Bacterial Overgrowth • SIBO is 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
- 30. SIBO • Normal and Abnormal Bacterial Distribution ©2003 RUSH University Medical Center
- 31. Intestinal Dysmotility • Motility may become so poor as to act as a obstruction to gut flow – i.e. chronic intestinal pseudoobstruction • PCI=air in gut wall due to loss of wall compliance and scaring. ©2003 RUSH University Medical Center
- 32. Colon • Colonic involvement in 20-50% of SSc patients • Loss of colonic contractions and motility • Colonic pseudoobstructions • Telangectasias • Bile acid injury ©2003 RUSH University Medical Center
- 33. Anorectum • Anorectal dysfunction in 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
- 34. Liver • Primary sclerosing cholangitis (PSC) ©2003 RUSH University Medical Center • 2-22% of patients • Slow progression to liver failure • Rare need for liver transplant • Treated with Actigall™; a synthetic bile Nodular regenerative hyperplasia: rare
- 35. Diagnostic Testing ©2003 RUSH University Medical Center
- 36. Assessment tools ©2003 RUSH University Medical Center
- 37. Treatment Options ©2003 RUSH University Medical Center
- 38. Esophageal Treatments • Lifestyle modification: 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, prucalopride • Reglan™, Motegrity ™ ©2003 RUSH University Medical Center
- 39. Long term PPI use concerns • Low magnesium levels (extremely rare) – Evidence is solid: check yearly magnesium levels • Kidney problems (very rare) – Evidence is solid: Check yearly kidney function • B12 deficiency – Evidence is fair: ( Check yearly B12 level over age 60. • Increased risk of COVID infection ©2003 RUSH University Medical Center
- 40. PPI issues; Unproven/refuted • Osteoporosis, bone fractures • Alzheimer's risk • Cardiac injury • Clostridium difficile colon infection • Pneumonia risk • Stomach or colon cancer risk • Iron deficiency ©2003 RUSH University Medical Center
- 41. Novel esophageal treatments • Endoscopic correction of GERD – Injection, plication, suture, polymer injection • New potassium competitive acid blockers • 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
- 42. Laparoscopic Anti-reflux Procedures • LARP: Surgical procedures to stop reflux by using the stomach to make an acid barrier. ©2003 RUSH University Medical Center
- 43. 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 • New! POEM. Endoscopic opening of the stomach valve ©2003 RUSH University Medical Center
- 44. Intestinal Treatments • Difficult and 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!). Metronidazole, trimethoprim-sulfamthoxazole – Prokinetics • Metocopramide occasionally effective • Erythromycin ineffective – Octreotide • Reduces symptoms, can help the skin • …but can delay stomach emptying ©2003 RUSH University Medical Center
- 45. FODMAP diet 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 ©2003 RUSH University Medical Center
- 46. FODMAP dieting • Seek out a dietician as it is not an easy diet to master. • Smartphone App – University of Monash FODMAP app ©2003 RUSH University Medical Center
- 47. 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
- 48. Anorectal Treatments • Stool bulking agents (soluble fiber/bran) • Antidiarrheal drugs – Immodium™, Lomotil™ • Tricyclic antidepressants – Amitriptyline, desipramine • Pelvic floor physical therapy • 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
- 49. New treatments • Prucalopride for motility of the small bowel and colon • Buspirone to reduce acid reflux by tightening the lower esophageal valve • Potassium competitive acid blockers to reduce acid • Probiotics for bloating ©2003 RUSH University Medical Center
- 50. Assessing Severity and Response ©2003 RUSH University Medical Center Curr Opin Rheumatol. 2013 November ; 25(6)
- 51. Summary • The GI tract 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 • Dysbiosis may play a role in cause and may be a site for future treatments • 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