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Spring/Summer 2007
Crohn’s Digest
New information about Crohn’s from Cleveland Clinic
Inflammatory Bowel Disease Can Affect the Entire Body
Unusual symptoms involving liver, joints, skin, eyes should be reported to physician
By Bret A. Lashner, M.D.
INSIDE:
Learn more about how
inflammatory bowel
disease affects many
parts of the body:
•
Eyes: Page 3
•
Joints: Page 4
•
Liver: Page 5
•
Skin: Page 5
Bret A. Lashner,
M.D.
Ulcerative colitis and Crohn’s disease are systemic diseases — meaning they
can affect all organs of the body, not just the gastrointestinal tract. While the
cause of these inflammatory bowel diseases (IBDs) is unknown, there is some
evidence that it may involve an autoimmune disease, where antibodies attack
the gastrointestinal tract. These same antibodies may cross-react with other
organ systems to cause inflammation in extra-intestinal manifestations
(EIMs), or sites in addition to the intestines.
IBD patients who experience other symptoms should talk with their physicians
to determine appropriate treatment. Below is a brief description of some possible EIMs. Articles in
this newsletter will provide more in-depth information for these conditions.
Liver function: PSC
About 5 percent of patients with IBD have abnormal liver function tests, and most of these patients
have no serious liver diseases associated with them. However, 2 percent of IBD patients (more
ulcerative colitis than Crohn’s disease) develop primary sclerosing cholangitis (PSC), scarring and
narrowing of the ducts that drain bile from the liver.
(Continued on page 2)
Cleveland Clinic
Digestive Disease Center
1
(Contiuned from page 1)
Inflammatory Bowel Disease
Can Affect the Entire Body
Symptoms of PSC are severe itching and
jaundice (yellow skin). Unfortunately,
successfully treating bowel inflammation
does not always improve inflammation in
the bile ducts. As there is no specific
treatment for PSC, patients should be
monitored closely by a hepatologist.
Patients with PSC have an increased risk
for developing colorectal cancer, so they
should have an annual colonoscopy.
Joint pain and swelling
Arthralgias (joint pains) and arthritis (joint
swelling) can occur in more than 20
percent of IBD patients; men and women
disease are more often affected with EN
than patients with ulcerative colitis, and
treatment consists of treating the bowel
disease.
PG is an ulcerating condition of the skin
that most often arises on the extremities or
the peristomal (skin surrounding the
stoma) area, although any region of the
skin can be affected. About 2 percent of IBD
patients will develop PG; patients with
Crohn’s disease are more than twice as
likely as ulcerative colitis patients to
develop PG. Only about half of patients
have PG symptoms that correlate with
bowel symptoms. Recent trials have shown
that infliximab (Remicade) is a very
effective therapy to treat PG.
Eye swelling, irritation
The main eye problems related to IBD are
episcleritis, scleritis and uveitis/iritis.
Patients with episcleritis look like they have
pink eye, but there is no infection. Eye
drops with steroids can treat episcleritis.
Patients with scleritis may experience
severe pain; this is a more serious condition that may require extensive treatment.
are equally affected. Typically, the large
joints, such as the knees and ankles, are
involved. Inflamed joints also may include
the joints in the spine (vertebrae) and the
sacro-iliac joints. As opposed to rheumatoid arthritis, the arthritis that comes from
IBD is not necessarily symmetric and is not
deforming. Usually, successful antiinflammatory treatment of the bowel will
improve joint symptoms.
Skin inflammation
The two most common skin manifestations of IBD are erythema nodosum (EN)
and pyoderma gangrenosum (PG). EN is
characterized by tender, raised red nodules
on the skin, usually on the shin. They are
associated with active bowel disease; in
fact, some patients may get an early
warning that bowel symptoms will develop
when EN appears. Patients with Crohn’s
2
Patients with uveitis (inflammation of the
cornea, cilia body and iris) and iritis
(inflammation of the iris leading to an
irregularly shaped pupil) may have eye pain
and blurred vision. Patients with these
conditions should see an ophthalmologist
for immediate treatment.
Other problems
In addition to the more commonly seen
conditions above, other problems related to
IBD, such as kidney stones, pancreatitis
and blood clots, are relatively uncommon.
Report any new symptoms to your physician, who can advise the most appropriate
course of action.
Crohn’s Digest is published bi-annually
by Cleveland Clinic’s Digestive Disease
Center to provide up-to-date information
about the department and its services.
The information contained in this publication is for educational purposes only
and should not be relied upon as medical advice. It has not been designed to
replace a physician’s medical assessment and medical judgment.
Please email us with questions and comments about this newsletter. We would
love to hear from you especially if you
have moved or changed phone numbers!
Drop us a line; this newsletter is for you!
Editorial Staff:
Editor
V. Maria Masina, RN, BSN, MSN
Editor
Jennifer Beling, RN, BSN
Managing Editor
Linda S. Libertini
Contact Information:
Ripka Family Database for
Crohn’s Disease Research
c/o Linda Libertini
Cleveland Clinic
9500 Euclid Ave./ W24
Cleveland, OH 44195
800-223-2273 extension 54148
[email protected]
Bret A. Lashner, M.D., is a specialist
in Cleveland Clinic’s Department of Gastroenterology and Hepatology. To schedule an
appointment to see him, please call
216.444.6536.
Eye Complications Occur in Patients with IBD
Correct diagnosis key to treatment
By Careen Y. Lowder, M.D., Ph.D.
Patients with inflammatory bowel disease (IBD) can experience eye complications such as episcleritis, scleritis and uveitis
before, after or in association with active IBD and is independent of the extent of bowel involvement.
EPISCLERITIS: Redness in one or both eyes
Episcleritis is the most common complication of
IBD. It is a noninfectious inflammatory condition
affecting the episcleral tissue, the layer that lies
between the conjunctiva and the sclera.
Careen Y. Lowder,
M.D., Ph.D.
To see a Cleveland
Clinic ophthalmologist,
please call
S Y M P T O M S . Episcleritis usually occurs as
redness in one or both eyes. There may be an area
of redness, or the redness may be spread throughout the surface of the eye. Occasionally, there may
be a nodule in the center of the inflamed area
(nodular episcleritis). Some patients may complain
of mild pain or tenderness to the affected region,
but this is rare.
216.444.2020.
T R E A T M E N T. Most cases resolve spontaneously
within a few weeks without treatment. Patients
who experience discomfort may benefit from
topical anti-inflammatory agents, lubricants and
cold compresses. Topical steroid drops will speed
resolution and decrease the tenderness. More
severe cases may require oral non-steroidal antiinflammatory agents (NSAIDs).
SCLERITIS: Serious condition with severe pain
Scleritis is a more serious, potentially blinding
condition that occurs in about 18 percent of IBD
patients. It is caused by inflammation of the deep
episcleral vessels (in between the conjunctiva and
sclera) leading to inflammation of the underlying
sclera, or the eye wall.
S Y M P T O M S . Patients may experience severe,
boring eye pain that can be associated with light
sensitivity, tearing and decreased vision. The
affected eye may take on a deep red, almost purple,
hue. Patients also may have scleral nodules
(nodular scleritis) and develop inflammation of the
cornea (peripheral keratitis) and inflammation in
the anterior chamber (secondary uveitis). In severe
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Digestive Disease Center
cases, the sclera may become very thin and
transparent. Scleritis, unlike episcleritis, requires
treatment.
T R E A T M E N T. Managing scleritis may involve
NSAIDs in addition to topical steroids. If the
inflammation is severe, or if NSAIDS fail to
suppress the inflammation, oral prednisone may
be necessary. In the most severe cases, the patient
may require immunosuppressive agents.
UVEITIS: Eye inflammation
Uveitis refers to inflammation of the uveal tract,
the layer between the sclera and the retina. Uveitis
has been reported in up to 17 percent of patients
with IBD. Women are at a higher risk of developing
uveitis than men.
S Y M P T O M S . An IBD patient may develop uveitis
in the front of the eye (anterior), causing redness,
eye pain, light sensitivity, or blurred vision. Uveitis
in the back (posterior) of the eye (less common)
affects the choroid and retina and can result in
vision loss. If the macula (central part of the retina)
is affected, central vision becomes impaired.
T R E A T M E N T. Patients with anterior uveitis (also
known as iritis) can be treated with dilating drops
and topical corticosteroids. Posterior uveitis may
be treated with injections of steroids. As with all
steroid treatment, patients must be carefully
monitored for development of secondary glaucoma. In steroid-dependent or resistant cases,
systemic immunosuppressive drugs may be
required.
Untreated, uveitis can lead to blindness.
As with every disease, discuss all new symptoms
with your physician as soon as possible, so that
your disease can be treated appropriately.
3
Arthritis, Osteoporosis
Connected to IBD
By Brian F. Mandell, M.D., Ph.D., F.A.C.R.
Patients with Crohn’s and ulcerative colitis (UC)
may experience musculoskeletal problems —
including several types of joint or muscle pain
and inflammation — either related to the
inflammatory bowel disease (IBD) or occasionally to a complication of IBD therapy.
Brian F. Mandell,
M.D., Ph.D.,
F.A.C.R.
To see a Cleveland
Clinic rheumatologist,
please call
216.444.5632.
Usually, the musculoskeletal condition arises
after the diagnosis of IBD. However, in about 15
percent of patients (perhaps higher in children),
it may begin prior to IBD diagnosis. It is believed
that most forms of arthritis related to IBD mirror
the disease’s flare-ups and remissions.
Arthritis types and characteristics
Several types of arthritis can occur in both
Crohn’s and UC patients. The most common
type affects one or both knees or ankles and
may affect other areas as well. Another type
affects many joints, including the wrists and
large finger knuckles, occasionally causing an
entire finger or toe to swell. Less commonly, the
joints of the spine, particularly the low back
(sacroiliac joints), are affected.
can be screened for osteoporosis with a bone
densitometry test; if it is present, appropriate
treatment can be prescribed. Calcium and
vitamin D supplements can be taken orally;
sometimes injections of vitamin D are necessary.
Exercise and treatment
Regular exercise, both aerobic and weightbearing, strengthens bones, maintains muscle
tone and provides more support to weightbearing joints, relieving pain symptoms for
musculoskeletal conditions and protecting
against osteoporosis.
Frequently, arthritis can be treated by increasing or adding medications that also treat IBD
such as sulfasalazine, Remicade or Humira.
Prednisone is effective, but use may be limited
to avoid the frequent complications of high-dose
steroid therapy. Sometimes lower doses of a
steroid are injected directly into an inflamed
joint to reduce incidence of exposure to the rest
of the body while still treating the arthritis effectively. Non-steroidal anti-inflammatory medications (NSAIDs, such as naproxen or ibuprofen)
may be used to relieve some joint pain and
inflammation, but in some cases, these medications can exacerbate IBD.
Symptoms include pain, stiffness and swelling
of the affected joints. The stiffness may be most
marked in the morning or after prolonged
periods of inactivity. Joints such as the hip,
shoulder and lower back do not often become
visibly swollen.
Osteoporosis and fibromyalgia common also
Osteoporosis occurs when bones do not get
adequate calcium to maintain their strength.
Patients with IBD can develop decreased bone
strength due to decreased absorption of calcium
(which can be worsened with steroid therapy) or
to decreased vitamin D levels. Decreased bone
strength can result in fractures, even without
major falls.
Osteoporosis is a potential complication of
steroid therapy that can be prevented. Patients
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Digestive Disease Center
Rarely, patients develop allergic reactions to
medications that cause inflammation in the
joints, fevers and/or rashes. When taken for long
periods, high-dose steroids can cause areas of
bone death affecting the hips and shoulders
that does not respond to anti-inflammatory
medications. It cannot be prevented, and the
pain may be severe enough to require surgical
therapy.
4
Skin Disease Common in Patients with Crohn’s
By Kenneth Tomecki, M.D., and Mandi P. Sachdeva, M.D.
Kenneth Tomecki,
M.D.
To see a Cleveland
Clinic dermatologist,
please call
216.444.5725.
Skin disease, relatively common in patients with
Crohn’s disease, may flare with disease activity or run
an independent course. The most common skin
problems are detailed below.
Crohn’s disease, so any patient with PG should be
Fissures. Fissures are most common in the perineum
Erythema nodosum. Erythema nodosum (EN) is a
(the region between the anus and the genital organs),
particularly the perianal area. Topical nitroglycerin or
injected botulism toxin are effective in relieving
symptoms.
characterized by tender, red lumps on the shins and is
Canker sores (aphthous ulcers). Canker sores are painful,
EN will resolve with control of the underlying disease,
shallow ulcers in the mouth. Treatment of the underlying Crohn’s disease can cure the sores, but symptomatic
relief may be necessary and includes gels or elixirs of
viscous lidocaine and/or topical corticosteroids.
although leg elevation, non-steroidal anti-inflammatory
evaluated for IBD. Treatment of PG can be difficult and
usually requires systemic corticosteroids in addition to
treatment of the underlying bowel disease.
often associated with joint pain, fever and a general
feeling of malaise. Repeated episodes of EN often
coincide with flares of Crohn’s disease. In most cases,
drugs (NSAIDs) and rest can help relieve symptoms.
Metastatic Crohn’s disease. This is a rare condition.
When it occurs, the legs, genitalia, perineum and
Pyoderma gangrenosum. Pyoderma gangrenosum (PG)
perianal region, and mouth tissues are most commonly
is a disease that causes tissue to become necrotic,
causing deep ulcers. PG typically begins as painful
blister that rapidly progresses to a noninfectious ulcer.
Most commonly found on the legs (although it can
occur anywhere), PG often precedes the onset of
affected. The lesions usually look like pimples, either
alone or in groups. In the mouth, it may have a “cobblestone” appearance. Although not as common as other
skin manifestations, this “cobblestoning” is specific for
Crohn’s disease.
Liver Disease Often Linked to IBD
By Nizar N. Zein, M.D.
Primary sclerosing cholangitis (PSC) is a disease in
(changes in mental status) or upper gastrointestinal
which the bile ducts (bile is a fluid that helps the body
bleeding from enlarged blood vessels in the esophagus
break down fat) inside and outside of the liver become
(esophageal varices).
inflamed and scarred. This scarring causes the bile
ducts to become clogged, preventing bile from flowing
out of the liver, damaging liver cells.
Nizar N. Zein,
M.D.
However, 25 percent of patients show no symptoms and
are diagnosed because of abnormalities detected during
routine examinations and tests. Diagnosis of PSC is
PSC is commonly associated with IBD as well as other
done with an X-ray procedure called a cholangiogram. A
diseases. In patients with PSC, 70 percent will have
liver biopsy is also performed to stage the disease.
ulcerative colitis and 5 to 8 percent will have Crohn’s.
Management of PSC
Symptoms and diagnosis
No specific medical therapy has been shown to reverse
The typical symptoms of PSC include fatigue, itching
or slow the progression of PSC. However, there are a
please call
and jaundice. Abdominal pain, weight loss and fever
number of ways to treat symptoms and the various
216.444.6126.
also common. Patients may have signs and symptoms
stages of the disease to improve quality of life. Liver
of advanced liver disease including ascites (fluid
transplant may be an option for some. Recurrence of
accumulation in the abdomen), encephalopathy
PSC after transplantation is rare.
To see a Cleveland
Clinic hepatologist,
5
Cleveland Clinic
Digestive Disease Center
Ripka Family Database for Crohn’s Disease Research Update:
What is a Database and Why is it Important?
A database is an electronically organized system of information,
such as statistics, that can be stored, managed, organized and
retrieved for analysis. Physicians and researchers use patient
databases to gain insight into disease trends, treatment management and outcomes/results.
Patient information is collected to help identify the best way to
treat the disease, as well as discover important ways to potentially
prevent the disease for future generations. Information that may be
relevant includes age at diagnosis, medications and quality-of-life
measurements. Through the accumulation of specific patient- and
disease-related data, researchers can compile data to report
outcomes, analyze trends and recognize best practices.
And, most importantly, researchers share these discoveries by
reporting on data through presentations and publishing research
articles. Sharing knowledge gleaned from clinical research can
ultimately affect medical decisions or help make improvements in
treating Crohn’s disease.
Last year, the Ripka Family Database underwent significant
software updates under the guidance of Principal Investigator
Scott Strong, M.D., in the Department of Colorectal Surgery, and
Co-Investigators J.P Achkar, M.D., and Bret Lashner, M.D., in the
Department of Gastroenterology. These changes have already
improved the way data are displayed and retrieved for the 1,370
enrollees in the database.
Patients who have consented to be in the Ripka Family Database
are motivated by a desire to help find answers related to Crohn’s
disease, and we thank them for their commitment. We look
forward to continuing our work toward improving the life of patients
with Crohn’s disease.
Sincerely,
The Ripka Family Database
for Crohn’s Disease Research Team
For more information, please call 216.445.4148.
MKT 07-DDC-011
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