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1 gastrointestinal manifestations of systemic sclerosis

"Gastrointestinal Manifestations of Systemic Sclerosis" presentation by Dr. Harald Schoeppner MD PhD. for the 12th annual Cheri Woo Scleroderma Education Seminar on March 9, 2013 hosted by Oregon Chapter of the Scleroderma Foundation.

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1 gastrointestinal manifestations of systemic sclerosis

  1. 1. Gastrointestinal Manifestations ofSystemic Sclerosis Harald Schoeppner, MD PhDLegacy Health Gastroenterology
  2. 2. Objectives Give an overview of Gastrointestinal involvement in patients with Systemic sclerosis Review some of the tests performed Review treatment options Emphasize on GERD (reflux disease)
  3. 3. Paul Klee (1879-1949)
  4. 4. Organ involvement in SSc GI involvement >90% Raynauds >90% Skin sclerosis >90% Arthritis/arthralgias >60% Pulmonary fibrosis >30% Renal involvement up to 20% Cardiac involvement 10% Literature, EUSTAR, dNSS database
  5. 5. Definitions Gastrointestinal (GI) tract: Several organs in continuity one-with the other whose main function is to digest food, absorb nutrients and excrete waste.
  6. 6. SSc affects the GI tract  New theory  Auto antibodies to myenteric neurons  M3R (anti- muscarinic 3 Ach R)
  7. 7. SSc affects the GI tract Any site can be affected Can affect pt with limited + diffuse SSc Can occur at any time Not always symptomatic Poor correlation with auto-antibodies Association between GI symptoms and quality of life scores Severe involvement in up to 6%DiCiaula A, BMC Gastro 2008; Forbes A, Rheumatol 2008; Thoua NM, Rheumatol 2010
  8. 8. SSc and the GI tractLIVER:PRIM BILIARY DYSPHAGIA/SCLEROSIS REFLUXEARLY SATIETY/ ANEMIABLOATING INTESTINAL BLEEDING MALABSORPTION/ WEIGHT LOSSPSEUDO-OBSTRUCTIONBACTERIALOVERGROWTH DIARRHEA/ CONSTIPATION FECAL INCONTINENCE
  9. 9. UCLA Scleroderma Clinical Trial Consortium GI Tract 2.0 Instrument Reflux Distention/bloating Fecal soilage Diarrhea Social functioning Emotional well-being Constipation Khanna. D Arthritis Rheum 2009
  10. 10. Mouth & Oropharynx Sicca symptoms (Sjogren’s)  Poor salivary function  Difficulty swallowing  Tooth cavities Mouth opening Minimal tongue involvement
  11. 11. Esophagus Most commonly affected organ Symptoms:  Heartburn  Regurgitation  Dysphagia  Chest pain  Atypical reflux symptoms  Hoarseness  Cough  ILD (interstitial lung disease)  Breathing problems (apnea)
  12. 12. Esophagus Poor lubrication Poor motility Absent sphincter barrier Normal Systemic sclerosis
  13. 13. Esophagus Complexity of GERD Sequelae:  Stricture  Ulcers  Barrett’s metaplasia  Esophageal cancer  Diverticula
  14. 14. Esophagus (treatment)  Lifestyle modification  No late meals (>4h)  Smaller meals  Elevate head of bed  Avoid “food stressors”  Orange, tomato juice  Spicy foods  Chocolate, coffee, tea  Lose weight if high BMI  Avoid alcohol  Avoid smoking
  15. 15. Esophagus - treatmentPROTON PUMP BLOCKER Other pharmacological tx “PPI”s – which is the right  H2 blockers one?  “Promotility drugs” Proper timing  Antacids Proper dosing  Avoid: Early initiation in all SSc  Calcium blockers Long term commitment  NSAIDs  Bisphonates Safety issues? Will prevent complications May help with ILD
  16. 16. GERD Is a Chronic Condition Likely to RelapsePatients in symptomatic remission (%) 100 No mucosal breaks LA Grade A 80 LA Grade B LA Grade C 60 40 20 0 0 1 2 3 4 5 6 Time after cessation of therapy (months) (months From Lundell LR, et al. Gut. 1999;45:172-180. 45:172-180
  17. 17. When do we do endoscopy? Patient not responding to treatment Complications  Intestinal bleeding  Anemia  Swallowing difficulties  Painful swallowing Cancer screening  Barrett’s
  18. 18. Cancer risk in SSc (Paris data) Barrett’s risk in SSc  Cancer risk  14/110 (12.7%)  50 individuals with  Dysplasia 3/14 Barrett’s  3 year follow up  4/46 developed HGD  1/50 developed cancer  18% no sx of GERD  Wipff, J 2005  Wipff, J 2011
  19. 19. Opportunities to intervene  Lifestyle  Medication  Screening  Surveillance  Ablation  Surgery
  20. 20. Stomach Roles:  Reservoir  Begins digestion  Produces acid  Allows absorption of iron and B12  Defense against ingested germs
  21. 21. SSc affects Stomach Impaired motility/contraction Symptoms related primarily to impaired emptying  Early satiety, bloating, regurgitation, belching, nausea, vomiting, ?pain  50% of patients with SSc have gastroparesis as measured, but fewer have symptoms
  22. 22. Scintigraphic frames at 0, 60, 120, and 180 min during infusion of saline (A) and GLP-1 (B) in one study subject during gastric emptying of solid meal. Näslund E et al. Am J Physiol Regul Integr Comp Physiol 1999;277:R910-R916©1999 by American Physiological Society
  23. 23. Stomach (treatment) Goals:  Improve symptoms  Improve nutritional status Methods:  Dietary changes  Medications  ?Pacemaker
  24. 24. Stomach (treatment) Gastroparesis Rx (early)  FDA Approved  Metoclopramide (reglan)  Erythromycin  Withdrawn from market  Cisapride  Not reviewed  Domperidone
  25. 25. Stomach GAVE “watermelon stomach” 10% incidence of Gastric Antral Vascular Ectasia Blood vessel involvement due to SSc May cause overt bleeding Causes iron deficiency anemia
  26. 26. Stomach GAVE treatment APC (Argon Plasma Coagulation) or other Cryotherapy Transfusions Iron replacements Cyclophosphamide  Several case reports  Indefinite length?
  27. 27. Small Bowel Anatomy  22-23 feet  3 regions Roles  Digestion of carbohydrates and protein and some fat.  Absorption of all nutrients  Absorption of water
  28. 28. Small Bowel Migrating Motor Complex 120 minute cycle 4 phases
  29. 29. Small Bowel SSc involves small bowel in 50-88% of pts  Only 6% have severe manifestations Symptoms vary (length of dz, extent dz)  Mild: bloating, fullness, belching  Severe: diarrhea, weight loss, malnutrition
  30. 30. Small Bowel Symptoms / pathology mostly due to impaired motility Slow transit Bacterial Overgrowth + Increased ‘fermentation’ Bile acid breakdown Excess Gas Diarrhea Bloating
  31. 31. Normal Loss of MMC; Decreased amplitudes
  32. 32. Bacterial Overgrowth Occurs 20% - 55% of patients with PSS Testing  Aspirates and culture  Hydrogen breath test
  33. 33. Malnutrition Screen for !  Lab tests Questionnaire  Hemoglobin BMI  Folic acid  Carotene level Weight loss  Prealbumin  - 1 – 2% in 1 week  Vit B12, Vit D, zinc  > 5% one month  > 7.5% 3 months  > 10% 1 year ? Depression
  34. 34. Small Bowel (treatment 1) Antibiotics  Types  Several effective agents  Tetracycline  Beware resistance  Doxycycline  Beware C. Diff colitis  Augmentin  Cycle agents  Non absorbable  Cephalexin + Flagyl preferred  Cipro  Nitazoxamid  Rifaximin
  35. 35. Small Bowel (treatment 2) Dietary  Less substrate to ferment and for bacteria (carbs)  Small, frequent meals  Consider FODMAP diet Improve motility  Domperidone  Erythromycin  Octreotide
  36. 36. Nutritional support Dietician Enteral nutrition  Jejunostomy Parenteral nutrition  TPN
  37. 37. Colon 3 Feet long Functions:  Absorb water  Concentrate feces  Excrete
  38. 38. Colon SSc symptoms:  Diarrhea  Constipation  Incontinence Mostly due to motility abnormalities of the colon and impaired anal sphincter
  39. 39. Colon Measuring transit timeDay 4 Day 7
  40. 40. Colon Intestinal ‘pseudo- obstruction’ (IPO)  Often involves small bowel  Signifies advanced stage  Avoid surgery (results in prolonged ileus)
  41. 41. How about colonoscopy? Colon cancer screening tool Investigate for intestinal bleeding Investigate for anemia Does nothing for constipation
  42. 42. Anal Sphincter Lax internal sphincter (neuropathic) Fibrotic sphincter (myopathic) Leads to incontinence and interfering with normal defecation.
  43. 43. Anal SphincterAno-rectal manometry New options 1.) Sacral stimulation 2.) Sphincter reconstruction
  44. 44. Colon (treatment) Constipation  Bulk-forming agents; fiber!  Water intake  Osmotic agents (avoid with IPO)  eg, PEG solutions  Stimulants (pro-motility)  Prunes, bisacodyl  Avoid narcotics, calcium blockers
  45. 45. Colon (treatment) Diarrhea  Investigate cause !  ? Overflow diarrhea  Infections (C. diff)  Bacterial overgrowth  Post-obstructive  Malabsorption  Celiac disease  Bile-acid diarrhea
  46. 46. Summary The GI tract may be affected to varying degrees Reflux is most commonly seen GI manifestations have impact on quality of life Treatment and diagnostic tools exist to help our patients Physicians knowledgeable in SSc are your best partners Treatment must be tailored to the patient’s individual needs
  47. 47. Thank you!
  48. 48. Diarrhea - approach Rule out overflow (Xray) Obtain stool tests (pathogens, c. diff) Obtain TTG (Sprue) Obtain fecal elastase, fecal leucocytes Trial of treatment for SIBO Cholestyramine if cholecytectomy Symptomatic treatment (fibers, loperamide) Trial of pancreatic enzymes
  49. 49. Distention, abdominal pain Exclude obstruction Consider gastroparesis (GES) Review medications ? DM Empiric trial of antibiotics for SIBO Dietician referral FODMAP Venting gastrostomy
  50. 50. Weight loss, nutrition Assess BMI Rule out depression Rule out malignancy Review with dietician Enteral/parenteral nutrition
  51. 51. Incontinence Assess frequency and stool consistency If lose: trial of Loperamide Testing: EUS, anorectal motility, defecography Biofeedback Low fiber diet Neuromodulation Sphincter augmentation
  52. 52. Constipation Establish: urge and emptying Drugs, thyroid function ?Prolapse Normal urge, infrequent: increase fiber No urge, not frequent: low fiber, supp, osmotic laxative Normal urge + emptying: stimulant Studies: colonoscopy, colonic transit Biofeed back, dietician, surgery

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