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"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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Gastrointestinal Gastrointestinal Manifestations of Manifestations of
Systemic Sclerosis Systemic Sclerosis
Harald Schoeppner, MD PhD
Legacy Health Gastroenterology
ObjectivesObjectives
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)
Paul Klee (1879-1949)Paul Klee (1879-1949)
Organ involvement in SScOrgan 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
DefinitionsDefinitions
Gastrointestinal Gastrointestinal (GI) tract:(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 tractSSc affects the GI tract
New theory Auto antibodies to
myenteric neurons M3R (anti-muscarinic
3 Ach R)
SSc affects the GI tractSSc 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 tractSSc and the GI tract
DYSPHAGIA/REFLUX
EARLY SATIETY/BLOATING
MALABSORPTION/WEIGHT LOSS
DIARRHEA/CONSTIPATION
PSEUDO-OBSTRUCTIONBACTERIALOVERGROWTH
FECALINCONTINENCE
ANEMIAINTESTINALBLEEDING
LIVER: PRIM BILIARYSCLEROSIS
UCLA Scleroderma Clinical Trial Consortium GI UCLA Scleroderma Clinical Trial Consortium GI Tract 2.0 InstrumentTract 2.0 Instrument
Reflux Distention/bloating Fecal soilage Diarrhea Social functioning Emotional well-being Constipation
Khanna. D Arthritis Rheum 2009
Mouth & OropharynxMouth & Oropharynx
Sicca symptoms (Sjogren’s) Poor salivary function Difficulty swallowing Tooth cavities
Mouth opening Minimal tongue involvement
EsophagusEsophagus
Most commonly affected organ
Symptoms: Heartburn Regurgitation Dysphagia Chest pain Atypical reflux symptoms
Hoarseness Cough ILD (interstitial lung disease) Breathing problems (apnea)
EsophagusEsophagus
Poor lubrication Poor motility Absent sphincter
barrier
Normal Systemic sclerosis
EsophagusEsophagus
Complexity of GERD Sequelae:
Stricture Ulcers Barrett’s metaplasia Esophageal cancer Diverticula
Esophagus (treatment)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 - treatmentEsophagus - treatment
PROTON PUMP BLOCKER “PPI”s – which is the right
one? Proper timing Proper dosing Early initiation in all SSc Long term commitment Safety issues? Will prevent complications May help with ILD
Other pharmacological tx H2 blockers “Promotility drugs” Antacids Avoid:
Calcium blockers NSAIDs Bisphonates
Pat
ien
ts i
n s
ymp
tom
atic
rem
issi
on
(%
)P
atie
nts
in
sym
pto
mat
ic r
emis
sio
n (
%)
100
80
60
40
20
00 1 2 3 4 5 6
Time after cessation of therapy (monthsTime after cessation of therapy (months)
No mucosal breaksNo mucosal breaks
LA Grade ALA Grade A
LA Grade BLA Grade B
LA Grade CLA Grade C
GERD Is a Chronic Condition GERD Is a Chronic Condition Likely to RelapseLikely to Relapse
From Lundell LR, et al. From Lundell LR, et al. Gut.Gut. 1999; 1999;45:172-18045:172-180.
When do we do endoscopy?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)Cancer risk in SSc (Paris data)
Barrett’s risk in SSc 14/110 (12.7%) Dysplasia 3/14
Wipff, J 2005
Cancer risk 50 individuals with
Barrett’s 3 year follow up 4/46 developed HGD 1/50 developed cancer 18% no sx of GERD
Wipff, J 2011
Opportunities to interveneOpportunities to intervene Lifestyle Medication
Screening
Surveillance
Ablation
Surgery
StomachStomach
Roles: Reservoir Begins digestion Produces acid Allows absorption of iron
and B12 Defense against ingested
germs
SSc affects StomachSSc 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
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
Stomach (treatment)Stomach (treatment)
Goals: Improve symptoms Improve nutritional status
Methods: Dietary changes Medications ?Pacemaker
Stomach (treatment)Stomach (treatment)
Gastroparesis Rx (early) FDA Approved
Metoclopramide (reglan)
Erythromycin
Withdrawn from market Cisapride
Not reviewed Domperidone
Stomach GAVE “watermelon Stomach GAVE “watermelon stomach”stomach”
10% incidence of Gastric Antral Vascular Ectasia
Blood vessel involvement due to SSc
May cause overt bleeding Causes iron deficiency anemia
Stomach GAVE treatmentStomach GAVE treatment
APC (Argon Plasma Coagulation) or other
Cryotherapy Transfusions Iron replacements
Cyclophosphamide Several case reports Indefinite length?
Small BowelSmall Bowel
Anatomy 22-23 feet 3 regions
Roles Digestion of
carbohydrates and protein and some fat.
Absorption of all nutrients
Absorption of water
Small BowelSmall Bowel Migrating Motor Complex 120 minute cycle 4 phases
Small BowelSmall 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 BowelSmall Bowel Symptoms / pathology mostly due to
impaired motility
Slow transitSlow transit
Increased ‘fermentation’Increased ‘fermentation’Bacterial OvergrowthBacterial Overgrowth
Excess GasExcess GasBile acid breakdownBile acid breakdown
DiarrheaDiarrhea BloatingBloating
++
NormalNormal
Loss of Loss of MMC;MMC;Decreased Decreased amplitudesamplitudes
Bacterial OvergrowthBacterial Overgrowth
Occurs 20% - 55% of patients with PSS Testing
Aspirates and culture Hydrogen breath test
MalnutritionMalnutrition
Screen for ! Questionnaire BMI Weight loss
- 1 – 2% in 1 week > 5% one month > 7.5% 3 months > 10% 1 year
? Depression
Lab tests Hemoglobin Folic acid Carotene level Prealbumin Vit B12, Vit D, zinc
Small Bowel (treatment 1)Small Bowel (treatment 1)
Antibiotics Several effective agents Beware resistance Beware C. Diff colitis Cycle agents Non absorbable
preferred
Types Tetracycline Doxycycline Augmentin Cephalexin + Flagyl Cipro Nitazoxamid Rifaximin
Small Bowel (treatment 2)Small Bowel (treatment 2)
Dietary Less substrate to ferment and for
bacteria (carbs) Small, frequent meals Consider FODMAP diet
Improve motility Domperidone Erythromycin Octreotide
Nutritional supportNutritional support
Dietician Enteral nutrition
Jejunostomy Parenteral nutrition
TPN
ColonColon
3 Feet long
Functions: Absorb water Concentrate feces Excrete
ColonColon
SSc symptoms: Diarrhea Constipation Incontinence
Mostly due to motility abnormalities of the colon and impaired anal sphincter
ColonColonMeasuring transit timeMeasuring transit time
Day 4Day 4 Day 7Day 7
ColonColon
Intestinal ‘pseudo-obstruction’ (IPO)
Often involves small bowel
Signifies advanced stage Avoid surgery (results in
prolonged ileus)
How about colonoscopy?How about colonoscopy?
Colon cancer screening tool
Investigate for intestinal bleeding
Investigate for anemia
Does nothing for constipation
Anal SphincterAnal Sphincter
Lax internal sphincter (neuropathic)
Fibrotic sphincter (myopathic)
Leads to incontinence and interfering with normal defecation.
Anal SphincterAnal Sphincter
Ano-rectal manometryAno-rectal manometry
New options1.) Sacral stimulation2.) Sphincter reconstruction
Colon (treatment)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
Colon (treatment)Colon (treatment)
Diarrhea Investigate cause !
? Overflow diarrhea Infections (C. diff) Bacterial overgrowth Post-obstructive Malabsorption Celiac disease Bile-acid diarrhea
SummarySummary
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
Thank you!Thank you!
Diarrhea - approachDiarrhea - 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
Distention, abdominal painDistention, abdominal pain
Exclude obstruction Consider gastroparesis (GES) Review medications ? DM Empiric trial of antibiotics for SIBO Dietician referral FODMAP Venting gastrostomy
Weight loss, nutritionWeight loss, nutrition
Assess BMI Rule out depression Rule out malignancy Review with dietician Enteral/parenteral nutrition
IncontinenceIncontinence
Assess frequency and stool consistency If lose: trial of Loperamide Testing: EUS, anorectal motility,
defecography Biofeedback Low fiber diet Neuromodulation Sphincter augmentation
ConstipationConstipation
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
Recommended