Medical Report: Digestive System (Gut, Gastro-intestinal) Involvement in Scleroderma - Scleroderma Foundation
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Medical Report:
Digestive System (Gut, Gastro-intestinal)
Involvement in Scleroderma
Updated January 2019
By Dinesh Khanna, MD, MS
Professor of Medicine
Director, University of Michigan Scleroderma Program
http://bit.ly/UMSclerodermaResearch
https://www.selfmanagescleroderma.com
1
A
fter the skin, the digestive
system is the most com Table 1: Frequency of GI symptoms
monly affected organ system (from 209 patients)
in people with scleroderma, affect
Prevalence
ing about 75 to 90 percent of all
Symptom (%)
patients (Table 1 and Figure 1).
The majority of people with gut • Heartburn 71
involvement experience symptoms • Trouble Swallowing 52
that interfere with their day-to-day • Bloating 80
activities and quality of life. • Diarrhea 51
The function of the gut is to push • Constipation 51
the food and liquid down from the • Fecal Incontinence 35
mouth to the large intestine (or
Scleroderma Foundation survey 2010
colon), extract and absorb nutrients,
and excrete the waste in the form of
stool. It does so by well-orchestrated mouth moist and use over-the- The esophagus is the area where
and rhythmic motions of the gut counter dry mouth products such most scleroderma patients have
muscles (also known as peristal- as Biotene® mouthwash and oral problems, and fortunately, it has
sis). The primary events that cause gel (more information available at been the area where the most
trouble in the scleroderma gut are www.biotene.com). Other available advances have been made in ther
due to a decrease in the blood sup- over-the-counter therapies include apy. The most common symptom
ply to the nerves, which are needed xylitol-containing products. Seeing is heartburn (sensation of burn
to stimulate the bowel. There is also a dentist on regular basis is recom ing behind the breast bone, also
some evidence that nerve damage mended. Prescription medications known as GERD [gastroesophageal
may occur due to an autoimmune such as Evoxac® and Salagen® may reflux disease]), which is due to
process. With decreased stimula- be helpful. the irritation of esophagus by acid
tion, there is progressive weakening regurgitating (backing up or reflux)
of muscle strength and tone and ESOPHAGUS (FOOD PIPE) from the stomach. Other symptoms
resultant slowing and discoordi- The esophagus transports food to may include dry cough at night,
nated motion of the gut. Virtually the stomach by coordinated con recurrent chest pain behind the
every gut symptom is the result of tractions of its muscular lining. breast bone, persistent hoarse voice,
weakening of the gut muscle. The This process is usually gradual and asthma (wheezing and shortness
weakening starts in the esophagus automatic and people are usually of breath), regurgitation of food
(food pipe) and stomach, and works not aware of it, except when they (expulsion of undigested food from
its way down to the small and large swallow something too large, try to the mouth), difficulty swallowing,
intestine. eat too quickly, or drink very hot or mouth ulcers and acid taste in the
This article will explain the very cold liquids. They then feel the mouth.
reasons for the gastrointestinal movement of the food or drink down Reflux is caused by weakening of
symptoms and discuss treatments the gut muscle valve between the
the esophagus into the stomach,
as well. stomach and esophagus, allowing
which may be an uncomfortable
sensation. acid to “splash” back up into the
OROPHARYNGEAL (MOUTH)
Approximately 20 percent of Figure 1: Gut Involvement
people with scleroderma develop
secondary Sjogren’s syndrome, a
syndrome associated with dry eyes Large
Intestine
and dry mouth. This may cause dif-
ficult swallowing, gum disease or Esophagus
cavities. If people have symptoms
Liver Small
of dry mouth, they should see a Intestine
dentist at least every six months
(for some, a visit every four months Stomach
may be required). In addition, use
sugar-free lozenges to keep your
2esophagus. The acid, while tracking
back into the food pipe, can some Table 2: Anti-Reflux Measures
times go into the lungs. Some clini • Keep head of the bed elevated, 6–8 inches (i.e. wedge pillow, blocks
cal studies have even suggested that under head of bed, electric bed).
the acid going into the lungs may • Take your biggest meal of the day at noon, small meals otherwise.
cause lung inflammation in sclero
• Avoid eating or drinking fluids two hours before bedtime.
derma. In addition, weakening of
the esophageal muscles themselves • Take frequent small meals (5–6 per day).
results in less efficient “milking” of • Do NOT use extra pillows. No tight garments around waist. (Both of them
the food down the esophagus, and may increase pressure over your stomach when bending at the waist
at times food can even get tempo causing reflux symptoms.)
rarily “stuck” in the esophagus and • Take anti-secretory and pro-motility agents (Table 2).
patients may need to vomit to clear • Stop smoking (if currently smoking).
the esophagus. • Avoid or minimize acid producing foods (fats, chocolate, coffee).
Diagnosis REMEMBER: Certain medications such as nifedipine (taken for Raynaud)
can weaken esophagus-stomach sphincter causing reflux symptoms. As
Diagnosis is based on symptoms.
always a balance of treatment should be achieved.
Most physicians will first treat
these symptoms with medications
that can help decrease the acid of the food pipe due to chronic acid improve reflux symptoms and also
production. If the symptoms con- damage). minimize the risk of aspiration.
tinue, tests such as barium swallow, Why do we need to treat reflux? Medications
endoscopy and esophageal manom-
The first and foremost reason to Proton pump inhibitors (PPIs)
etry can be performed. The barium
treat reflux is to relieve symptoms The PPIs are medications that sup-
swallow requires the person to swal-
and improve your quality of life. press acid production. The goal of
low a liquid, which contains barium
As mentioned before, acid reflux the treatment is to completely stop
mixed in a drink, and then take an
can also lead to lung inflammation the symptoms of heartburn because
X-ray of their esophagus and stom- continuing symptoms, even if occa-
and even scarring of the lung tissue.
ach. Because these organs are nor- sional, reflect ongoing acid produc-
Continuous high acid production
mally not visible on X-rays, barium tion. To achieve this, higher than
for a long period of time, when
temporarily coats the lining of the recommended doses of PPIs are
the esophagus-stomach sphincter
esophagus, stomach and intestine, sometimes used (Table 3). Another
is weak, can cause inflammation
making the outline of these organs piece of advice for people with
and scarring of the esophagus
visible on the X-ray pictures. This scleroderma and reflux is to NEVER
(also known as stricture), which
process helps to look for the motility leave home without the PPIs, as
can lead to Barrett esophagus (a
or gut muscle integrity and ulcers missing a couple of doses can cause
precancerous condition).
(breakdown in the lining of the an increase in acid production and
Periodic endoscopic examinations
gut) in the esophagus and stomach. rebound of severe symptoms. PPIs
to look for early warning signs of
Esophageal manometry requires a should be preferably taken 30 min
cancer are generally recommended utes before breakfast and dinner
small tube be put through the nose
for people who have Barrett (if needed).
into the esophagus. The tube
esophagus, an approach referred Recent studies have indicated
is built to sense pressure, so it can
to as surveillance. an increased risk of diarrhea (may
measure sequential muscle con-
Therapy rarely cause Clostridium difficile
tractures (the “milking” motion),
infection), osteoporosis, dementia,
and evaluate the motility and func- The treatment of acid reflux and chronic kidney diseases with
tion of the esophageal muscles and requires both lifestyle changes and long term use of PPIs. Therefore,
the esophagus-stomach sphincter. medications that can you should be aware of these and
Endoscopy requires a gastroenter- 1. Decrease acid production. discuss with your physician. The
ologist to put a small camera at 2. Increase the motility of the gut benefits still outweigh risks in many
the end of a small tube (the endo- muscle. people with scleroderma. Some
scope) through your mouth into the people try certain over the coun
esophagus and stomach to look for Lifestyle changes
ter medications, such as Zantac®,
damage caused by the acid, such Table 2 goes over the lifestyle Pepcid® (known as H2 blockers)
as ulcers and strictures (narrowing changes that may help significantly which may provide temporary relief,
3the reflux symptoms otherwise the blood vessels can cause anemia
Table 3: Medications damage to the esophagus will con- (low red blood cell count). The per
Some of the medications used to treat gut tinue and the stricture will return. son may or may not have stomach
problems in scleroderma:
The stricture, in fact, may require symptoms and may only feel VERY
ACID-SUPPRESSING AGENTS
Proton-pump inhibitors repeated dilations to keep it open, tired and fatigued.
• Prilosec® (omeprazole) 20-40 mg 1 to 2 times per day but the need to redilate usually
• Prevacid® (lansoprazole) 15-30 mg 1 to 2 times per day
• Aciphex® (rabeprazole) 20 mg 1 to 2 times per day decreases over time. Surgery due to Diagnosis
• Protonix® (pantorazole) 40 mg 1 to 2 times per day “non-functioning” esophagus should
• Nexium® (esomeprazole) 20-40 mg 1 to 2 times per day
Gastroparesis can be diagnosed
• Dexilant® (dexilansoprazole) 30-60 mg once per day be the last resort as it often does using the barium study, as dis-
H-2 blocker (Over the counter) not work very well. This should only cussed in the esophagus section.
• Pepcid®, Tagamet®, Zantac®, Axid® I tablet at night and
as needed if on maximum doses of proton-pump inhibitors
be considered after a lengthy discus Also, a gastric emptying study can
PRO-MOTILITY AGENTS
sion with your rheumatologist. The be performed in which a person
• Reglan® (metaclopromide) 10 mg 3 to 4 times per day† procedure should be performed by eats a meal (e.g. a sandwich or
• E-Mycin® (erythromycin) 250-333 mg 3 to 4 times per day
• Domperidone®* 10-20 mg 3 to 4 times per day surgeons who have experience in
egg) which contains a very small
• Sandostatin® (octreotide)** 50 mcg, 1 to 2 times per day scleroderma.
BACTERIA SUPPRESSING ANTIBIOTICS
amount of radiolabeled material
• Amoxil®, Trimox® (amoxicillin) 500 mg 3 times per day STOMACH (that is safe) followed by X-rays
• Augmentin® (amoxicillin/ clavulanate) 500/125 or 875/125
mg 2 times per day Stomach symptoms in scleroderma of their stomach. The doctors are
• Cipro® (ciprofloxacin) 500 mg 3 times per day trying to see how long it takes
• Flagyl® (metronidazole) 500 mg 3 times per day are due to slow emptying of the food
• Doxy-100® (doxycyline) 100 mg 2 times per day
(also known as gastroparesis) into the food to empty into the small
• Bactrim® Double Strength 1 tablet 2 times per day
• (trimethoprim-sulfamethoxazole) the small intestine. The retention of bowel. In scleroderma, the food and
• Xifaxan® (rifaximin) 400 mg up to 3 times per day
• Neomycin 500 mg 2 to 4 times per day food in the stomach leads to a sen- radiolabeled material remain in
LAXATIVES@ sation of nausea, vomiting, fullness, the stomach longer than normal
• Colace® (Docusate sodium) 100 mg 1 to 2 times per day
or bloating sensation and disten (sometimes hours) before emptying
• Lactulose 15-30 ml 1 to 2 times per day
• Milk of magnesia 30-60 ml once per day tion. In some people with sclero- completely. In a person with normal
• Senna® (sennosides) 1 capsule 1 to 2 times per day
• Amitizia® (Lubiprostone) 8-24 ug 2 times per day derma, the stomach can also have stomach function, approximately 50
† has a black box warning. Rare cases of tardive dyskinesia, a rare telangiectasias (dilated blood ves percent of food is remaining in the
movement disorder with prolonged use.
sels) lining the walls of the stomach. stomach after 90 minutes. Other
This is also known as “watermelon radiographic test includes a wireless
but are usually not very effective in
stomach” due to its appearance on motility capsule which is a single-
controlling the reflux symptoms and
take PPIs as “on-demand” therapy. endoscopy. Slow and intermittent use, orally ingested, non-digestible,
Some physicians use combinations or rapid bleeding from these dilated capsule with data recorder. This can
of the PPIs and H2 blockers.
The prokinetic medications (those Figure 2: Watermelon Stomach
that improve motility of the gut
muscles) can also be used if the
symptoms of reflux continue even
after reaching maximum doses of
PPIs (Table 3). The prokinetic medi-
cation should be taken one hour
before the meal so the medication is
“on board” when one eats.
Surgery
As mentioned before, the treat-
ment of the stricture (which is
the scarring of the esophagus due
to repeated acid damage) may
require dilatation of the narrowing
of esophagus during an endoscopy
procedure. Progressively longer
tubes are gently passed through the
narrowed areas to stretch it and
open it up. This helps improve the
downward motion of the food. This
MUST be followed by good control of
4assess the gastric emptying time
but also help with monitoring small
and large bowel motility as dis
Three common causes of
cussed below.
Diagnosis of “watermelon
weight loss or inability to gain
stomach,” with its characteristic weight are: gastroparesis, small
appearance, can be made by an
experienced gastroenterologist using bowel bacterial overgrowth and
endoscopy (Figure 2).
untreated depression.
Treatment
The treatment of gastroparesis
symptoms of diarrhea with foul people may require longer courses.
requires similar measures as
smelling stools, which may be oily Any antibiotic may be tried based
mentioned in Table 2, and use of
or hard to flush. on previous response, side effects
prokinetic medications mentioned
Another small bowel symptom and coverage by the insurance.
in Table 3. For “watermelon stom-
that occurs in scleroderma is pseudo Rifaximin and neomycin are oral
ach,” use of an argon laser to coagu
(false)-obstruction. In this condition, antibiotics that have minimal sys
late or burn the bleeding vessels
the bowel is not physically blocked temic absorption. Usually a person
can lead to improvement in the ane
as in true bowel obstruction but has notices improvement in the symp
mia and symptoms. Patients may
just “had enough” and stops work- toms after a course of treatment
require multiple laser treatments
ing. This is caused by weakening and may notice a slow weight gain.
(every two to four weeks) before the
of the gut muscle. The patient com- Very often, the treatment has to be
bleeding stops completely.
plains of belly pain, distention, vom- repeated every month or so to keep
iting, and inability to “pass gas.” the bacteria level low. Occasionally
SMALL INTESTINE (SMALL treatment must be continuous
BOWEL) Diagnosis and some physicians use different
The small bowel is the place Bacterial overgrowth is usually antibiotics every month to prevent
where we gain most of the nutrition diagnosed based on symptoms and the bacteria from becoming resis
from our food. The food is pushed treated accordingly. If the symp- tant. Pro motility agents (Table 3)
from the stomach into the small toms persist after treatment, a can also help. Patients may also
bowel by the rhythmic motions of breath test can be performed. In obtain symptomatic relief in the
the gut. Once in the small bowel, this, a person ingests sugar (lac bacterial overgrowth syndrome by
the nutrients and vitamins are tose), which is broken down by the regular use of yogurt containing
extracted from the food and the bacteria in the small bowel and the bowel-friendly bacteria. Probiotics
waste is propelled into the large by-product, hydrogen, is detected are bacteria that have a beneficial
bowel and excreted as stool. The in the breath. The physician should effect in the prevention and treat-
symptoms of bowel involvement are also evaluate vitamin deficiencies ment of gut problems when they are
due to lack of muscle tone, leading such as Vitamin B12, D and folic ingested. Florastor™, Culturelle™,
to stagnation of food in the small acid. and Align™ are three standardized
and large bowel. Diagnosis of pseudo-obstruction choices (one or two doses daily) that
In the small bowel, the symptoms requires a plain X-ray and a CT can be used. Care should be taken
are similar to gastroparesis (bloat scan of the abdomen. The CT scan not to use probiotics if you are tak-
ing sensation and distention, nau detects whether the symptoms are ing immunosuppressive therapies
sea and vomiting), but abdominal due to a tired “gut,” one that needs as probiotics can rarely cause blood
pain may also occur. In addition, rest, or there is something obstruct- infection from live bacteria. Also,
due to stagnation of food, the bacte ing the small bowel. there is lack of high quality pub
ria, which normally reside in small lished data to support their efficacy.
quantities in the small bowel, can Treatment Sandostatin® (octereotide) has
multiply in the food causing “bacte- Bacterial overgrowth is treated been successfully used during
rial overgrowth syndrome.” These using antibiotics to reduce the attacks of pseudo-obstruction, and
bacteria compete with nutrients overgrowth of bacteria in the small if attacks are frequent, can some-
and vitamins causing vitamin defi- bowel. This is achieved by using times be used on a regular basis to
ciencies, weight loss, and inability antibiotics for 10 to 14 days at prevent any further attacks. Once
to gain weight. There may also be a time (Table 3), although some treated, a person should take
5prescribed multivitamins to prevent the same as the common diverticu body. About 10 percent of sclero-
or treat vitamin deficiencies. losis that you often hear about). derma patients may have liver
Pseudo-obstruction usually Diverticulitis usually presents with involvement, known as primary
requires hospitalization and com belly pain and fever. People can also biliary cirrhosis. If this uncommon
plete rest of the small bowel. The get diarrhea due to impacted stool. complication occurs, it usually
person is given nutrients through occurs 10 to 15 years after the onset
Diagnosis
the veins along with pro-motility of scleroderma. Some patients may
agents. Diagnosis usually starts with symp-
develop biliary cirrhosis before a
Venting is also performed when toms related to the colon. A barium
diagnosis of scleroderma. Symptoms
needed. “Venting” means to put a study can help look at the large
usually consist of itchy skin and
small surgical tube into the jeju- bowel. Other radiographic tests
fatigue. The diagnosis can be made
num (small intestine) to aspirate include radiopaque markers and
by blood tests for anti-mitochondrial
out the air. This keeps the patient motility capsule.
and anti-smooth muscle antibody
from becoming severely distended Treatment and the treatment consists of doses
while in treatment. Total paren- of ursodeoxycholic acid.
Treatment for constipation requires
teral nutrition (TPN) [high energy
liberal use of water and laxatives
liquid diet through the veins] may DIET AND SCLERODERMA
that stimulate the nerve endings in
be prescribed by your physician to Eating Well with Scleroderma
the gut wall, which make the mus-
give your bowel a prolonged rest (www.scleroderma.org/eatingwell)
and help provide you with essen- cles in the intestine contract with
more force. A high fiber diet without provides important information
tial nutrition and energy. In many
a laxative is not helpful as it just regarding diet that may help or
patients, the bowel slowly recovers
adds to the amount of waste the worsen gastrointestinal symptoms.
after two to three months, and peo
ple can go back to eating bowel is struggling with. The person
HOW TO MEASURE GUT
regular food. can take a laxative every other day
INVOLVEMENT AND SEVERITY IN
to maintain a healthy bowel regi-
DAY-TO-DAY CARE
LARGE INTESTINE (COLON) men. Other laxatives are included
in Table 3. Researchers have developed
The main function of the large
a 34-item questionnaire that
bowel is to reabsorb water and salts
RECTUM can assess gut involvement
that have been secreted by the rest
Stool incontinence (symptom of and severity. The question
of the gut. This helps the forma-
accidentally soiling the underwear naire is called the University of
tion of stools and helps to move the
before being able to get to a bath- California- Scleroderma Clinical
waste along. In scleroderma, there
room) occurs in up to a third of peo- Trial Consortium Gastrointestinal
is, as is the case with the rest of the
ple with scleroderma. This is due to Instrument. It has seven scales: five
gut, weakening of the gut muscles
the weakening of the rectal muscle scales assess symptoms - reflux,
and impaired motility. This can lead
and poor control over the rectal distention/bloating, diarrhea, fecal
to constipation. Constipation means
different things to different people. sphincter. Anorectal manometry can soilage (or rectal incontinence), and
For many people, it simply means assess function of this sphincter. constipation, and two scales assess
infrequent stools. Medically speak Biofeedback therapy may be helpful the impact of gut symptoms on the
ing, constipation usually is defined by improving voluntary squeezing emotional well-being and social
as fewer than three bowel move of the rectal muscle. Major sclero- functioning. All scales are scored
ments per week. Severe constipation derma centers offer classes to teach from 0 (no symptoms or impact on
is defined as less than one bowel people this technique. Surgery by quality of life) to 3 (severe symp
movement per week. This is usually an experienced surgeon might also toms or poor quality of life) except
associated with pain and a feeling of be helpful. Preliminary studies diarrhea and constipation scales
incomplete emptying of the bowel. have shown promise of sacral nerve that ranges from 0-2 and 0- 2.5,
Also, one may get wide-mouthed stimulation (nerves that control rec- respectively. The questionnaire
diverticula (large pouches) in the tal tone using a device [Interstim®) takes approximately 5-7 minutes to
colon. These do not ordinarily cause in decreasing episodes of rectal complete and is available free online
any symptoms. However, on rare incontinence. at http://bit.ly/sctcgit.
occasions, the stool can get impact The log-in takes less than a min
ed in them causing inflammation of LIVER ute and asks optional questions
the diverticula, called diverticulitis The liver plays an important role regarding your name and e-mail
(this is similar to, but not exactly in the detoxification of drugs in our address. These questions are asked
6so you may receive future e-mails
on new developments regarding this Table 4: GI Questionnaire
questionnaire and invitations for • High Reflux scale score➔ anti-reflux, PPIs, and promotility (if needed)
future studies. In addition, the log-
in screen asks for your age, gender, • High Distention/Bloating scale score➔ Consider delayed gastric emptying
or bacterial overgrowth
and if you have scleroderma. The
– PLUS Diarrhea➔ trial of antibiotics
questionnaire is available in 34 lan
– No Diarrhea➔ trial of promotility agents
guages. The questionnaire can be
used by a person to assess their GI • High Constipation scale score➔ stimulant laxatives, good bowel regimen
health and monitor their response • Fecal soilage➔ appointment with a colorectal surgeon
to treatment. Each scale is pre
sented as a green bar (none-to-mild has severe reflux symptoms, mod promotility agents. The person can
symptoms), a yellow bar (moderate erate diarrhea, mild distention/ assess improvement or change in
symptoms), or red bar (severe-to- bloating, with no fecal soilage and his/ her symptoms by completing
very severe symptoms). constipation. the questionnaire at regular inter
Table 4 provides an example These GI symptoms are lead vals. A red bar should also alert the
of an approach in using the scale ing to severe emotional and social person to notify their physician of
scores for treatment purposes. As distress. Treatment should include worsening symptoms.
an example, the patient in Figure 3 anti-reflux measures, PPIs and
Figure 3: GI Questionnaire
The UCLA SCTC GIT 2.0 Questionnaire
For an interactive version of the questionnaire please visit
http://bit.ly/sctcgit
7The Scleroderma Foundation’s mission is three-fold:
a To help patients and their families cope with scleroderma through mutual support programs,
peer counseling, physician referrals, and educational information.
a To promote public awareness and education through patient and health professional semi
nars, literature, and publicity campaigns.
a To stimulate and support research to improve treatment and ultimately find the cause and
cure of scleroderma and related diseases.
300 Rosewood Drive, Suite 105
Danvers, MA 01923
Phone: 978-463-5843
Fax: 978-777-1313
Info Line: 800-722-HOPE (4673)
Email: sfinfo@scleroderma.org
Website: www.scleroderma.org
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www.twitter.com/scleroderma
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Text and logo © 2019, The Scleroderma Foundation, Inc.
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