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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.
1.) behind every disease there is a patient 2.) hope: most productive period of his life 3.) one cannot be defined by the disease
Dr. Lundell and colleagues conducted a study of patients who were diagnosed with reflux disease. Their symptoms were relieved and erosive esophagitis (if present) was healed with a course of PPI therapy. 1 Six months following treatment, symptomatic relapse occurred in an average of 83% of patients regardless of the status of their esophageal mucosa at the time of relapse. It is also important to note that the majority of patients who relapse do so within three months of stopping therapy. 2 References: 1. Lundell LR, et al. Gut . 1999;45:172-180. 2. Vakil NB, et al. Aliment.Pharmacol Ther. 2001;15:927-935.
Most of the threat in SSc are relaed to smoking, lung cancer
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.
Initial treatment: 14 days course of antibiotics, if relapse then 1 st 10 days every month, then add prokinetics (reglan avoid, Dom 10-20 Q6, Emycin at night 200mg at night, Octreotide 50-100yg qhs, long acting 2omg q month, octreotide + emcyin)
1 gastrointestinal manifestations of systemic sclerosis
Gastrointestinal Manifestations ofSystemic Sclerosis Harald Schoeppner, MD PhDLegacy Health Gastroenterology
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)
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
Definitions Gastrointestinal (GI) tract: Several organs in continuity one-with the other whose main function is to digest food, absorb nutrients and excrete waste.
SSc affects the GI tract New theory Auto antibodies to myenteric neurons M3R (anti- muscarinic 3 Ach R)
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
SSc and the GI tractLIVER:PRIM BILIARY DYSPHAGIA/SCLEROSIS REFLUXEARLY SATIETY/ ANEMIABLOATING INTESTINAL BLEEDING MALABSORPTION/ WEIGHT LOSSPSEUDO-OBSTRUCTIONBACTERIALOVERGROWTH DIARRHEA/ CONSTIPATION FECAL INCONTINENCE
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
Esophagus Complexity of GERD Sequelae: Stricture Ulcers Barrett’s metaplasia Esophageal cancer Diverticula
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
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
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
When do we do endoscopy? Patient not responding to treatment Complications Intestinal bleeding Anemia Swallowing difficulties Painful swallowing Cancer screening Barrett’s
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
Opportunities to intervene Lifestyle Medication Screening Surveillance Ablation Surgery
Stomach Roles: Reservoir Begins digestion Produces acid Allows absorption of iron and B12 Defense against ingested germs
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
Stomach (treatment) Gastroparesis Rx (early) FDA Approved Metoclopramide (reglan) Erythromycin Withdrawn from market Cisapride Not reviewed Domperidone
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
Stomach GAVE treatment APC (Argon Plasma Coagulation) or other Cryotherapy Transfusions Iron replacements Cyclophosphamide Several case reports Indefinite length?
Small Bowel Anatomy 22-23 feet 3 regions Roles Digestion of carbohydrates and protein and some fat. Absorption of all nutrients Absorption of water
Small Bowel Migrating Motor Complex 120 minute cycle 4 phases
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
Small Bowel Symptoms / pathology mostly due to impaired motility Slow transit Bacterial Overgrowth + Increased ‘fermentation’ Bile acid breakdown Excess Gas Diarrhea Bloating
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