Clinical-Manifestations-Diagnosis and Prognosis of Crohn Disease

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Clinical manifestations, diagnosis and prognosis of Crohn disease in adults
Authors: Mark A Peppercorn, MD, Sunanda V Kane, MD, MSPH

Section Editor: Paul Rutgeerts, MD, PhD, FRCP
Deputy Editor: Kristen M Robson, MD, MBA, FACG
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Jun 2018. | This topic last updated: Jun 07, 2018.
INTRODUCTION — Crohn disease (CD) is a disorder of uncertain etiology that is characterized by

transmural inflammation of the gastrointestinal tract. CD may involve the entire gastrointestinal tract
from mouth to the perianal area:
● Approximately 80 percent of patients have small bowel involvement, usually in the distal ileum,
with one-third of patients having ileitis exclusively.
● Approximately 50 percent of patients have ileocolitis, which refers to involvement of both the
ileum and colon.
● Approximately 20 percent have disease limited to the colon. In contrast to rectal involvement in
patients with ulcerative colitis, one-half of CD patients with colitis have sparing of the rectum.
● Approximately one-third of patients have perianal disease.
● Approximately 5 to 15 percent have predominant involvement of the mouth or gastroduodenal

area, while fewer patients have involvement of the esophagus and proximal small bowel. This
topic review will discuss the clinical manifestations, diagnosis, differential diagnosis, and natural
history of CD. The management of this disorder is discussed separately. (See "Overview of the
medical management of mild (low risk) Crohn disease in adults" and "Overview of medical
management of high-risk, adult patients with moderate to severe Crohn disease".)
CLINICAL MANIFESTATIONS — The clinical manifestations of Crohn disease (CD) are more
variable than those of ulcerative colitis. Patients can have symptoms for many years prior to diagnosis
[1-3]. Fatigue, prolonged diarrhea with abdominal pain, weight loss, and fever, with or without gross
bleeding, are the hallmarks of CD [4].
Abdominal pain — Crampy abdominal pain is a common manifestation of CD, regardless of disease
distribution. The transmural nature of the inflammatory process results in fibrotic strictures. These
strictures often lead to repeated episodes of small bowel, or less commonly colonic, obstruction. A
patient with disease limited to the distal ileum frequently presents with right lower quadrant pain.
https://www.uptodate.com/contents/clinical-manifestations-diagnosis-and-prognosis-of-cro... 7/19/2018
Occasionally, patients will have no clinical manifestations of CD until luminal narrowing causes
constipation and early signs of obstruction with abdominal pain.
Diarrhea — Diarrhea is a common presentation, but often fluctuates over a long period of time. A
history of prolonged diarrhea without bleeding but with other features suggestive of inflammatory
bowel disease (IBD) (eg, skin, eye, or joint problems, or a family history of IBD) should suggest the
diagnosis of CD. Diarrhea associated with CD may have multiple causes, including:
● Excessive fluid secretion and impaired fluid absorption by inflamed small or large bowel
● Bile salt malabsorption due to an inflamed or resected terminal ileum
● Steatorrhea related to loss of bile salts
● Small intestinal bacterial overgrowth
● Overlapping irritable bowel syndrome
● Enterocutaneous fistulas causing bypass of portions of absorptive surface area
Bleeding — Although stools frequently reveal the presence of microscopic levels of blood (eg,
positive guaiac or immunochemical test), gross bleeding is less frequent than in ulcerative colitis. An
exception to this are some patients with Crohn colitis.
Fistulas — Transmural bowel inflammation is associated with the development of sinus tracts. Sinus
tracts that penetrate the serosa can give rise to fistulas. Penetration of the bowel wall most often
presents as an indolent process, and not as an acute abdomen.
Fistulas are tracts or communications that connect two epithelial-lined organs. Common sites for
fistulas connect the intestine to bladder (enterovesical), to skin (enterocutaneous), to bowel
(enteroenteric), and to the vagina (enterovaginal).
The clinical manifestation of the fistula depends upon the area of involvement adjacent to the
diseased bowel segment.
● Enteroenteric fistulas may be asymptomatic or present as a palpable mass
● Enterovesical fistulas lead to recurrent urinary tract infections, often with multiple organisms, and
to pneumaturia
● Fistulas to the retroperitoneum may lead to psoas abscesses or ureteral obstruction with
hydronephrosis
● Enterovaginal fistulas may present with passage of gas or feces through the vagina
● Enterocutaneous fistulas can cause bowel contents to drain to the surface of the skin
In a population-based study of 169 patients with CD, 12 patients (7 percent) had evidence of a fistula
of any type at least 30 days prior to the diagnosis of CD [5]. (See "Perianal complications of Crohn
disease", section on 'Perianal fistula'.)
Phlegmon/abscess — All sinus tracts do not lead to fistulas. Sinus tracts may present as a
phlegmon, a walled off inflammatory mass without bacterial infection that may be palpable on physical
examination. Ileal involvement is suggested by a mass in the right lower quadrant.
Some sinus tracts lead to abscess formation and an acute presentation of localized peritonitis with
fever, abdominal pain and tenderness. Diffuse peritonitis due to abscess perforation is a rare but
recognized complication of CD.
Perianal disease — Symptoms and signs related to perianal disease occur in more than one-third of
patients with CD and may dominate the clinical picture. These include perianal pain and drainage from
large skin tags, anal fissures, perirectal abscesses, and anorectal fistulas. (See "Perianal
complications of Crohn disease".)
Malabsorption — Bile acids are normally absorbed by specific receptors in the distal ileum. Bile salt
malabsorption occurs when more than 50 to 60 cm of terminal ileum is diseased or resected.
Unabsorbed bile salts enter the colon resulting in a secretory or "bile salt" diarrhea. (See "Clinical
features and diagnosis of malabsorption".)
Increased synthesis partially compensates for the loss of bile salts. However, compensation is
inadequate with disease or resection of more than 100 cm of the terminal ileum, leading to depletion
of the bile salt pool and fat malabsorption. Thus:
● A watery diarrhea may result related to the effects of bile acids on colonic water and electrolyte
absorption.
● Steatorrhea is possible with a more severe degree of bile acid malabsorption, since the
concentration of bile acids required for micelles for fat absorption is impaired.
● Steatorrhea may also result from bacterial overgrowth from small bowel strictures, enterocolonic
fistulas, and extensive jejunal disease.
Steatorrhea can lead to severe malnutrition, clotting abnormalities, osteomalacia, and hypocalcemia,
which may cause tetany.
Other gastrointestinal involvement — The clinical manifestations of other sites of gastrointestinal

involvement in CD are variable and infrequent. Examples include:
● Severe oral involvement may present with aphthous ulcers or pain in the mouth and gums.
● Esophageal involvement may present with odynophagia and dysphagia.
● Gallstones may form since the reduction in the bile acid to cholesterol ratio makes bile more
lithogenic and abnormalities in bilirubin metabolism predispose to pigment stones [6].
Gastroduodenal CD, seen in up to 15 percent of patients, may present with upper abdominal pain,
nausea, and/or postprandial vomiting [7]. The clinical features of gastroduodenal CD may be similar to
those of peptic ulcer disease or gastric outlet obstruction.
The distal antrum of the stomach and duodenum are the most commonly affected upper
gastrointestinal sites in patients with CD. There are patients with very mild and limited upper
gastrointestinal involvement, with or without symptoms, while others with significant symptoms have
severe disease characterized by deep ulcers and longer segments of intestinal involvement. Patients
with extensive upper gastrointestinal tract involvement tend to have a more aggressive disease course
compared to patients without gastroduodenal involvement.
Systemic symptoms — Fatigue is a common feature of CD. Weight loss is often related to
decreased oral intake since patients with obstructing segments of bowel feel better when they do not
eat. Weight loss may also be related to malabsorption.
Fever occurs less frequently and may be due to the inflammatory process itself or may be the result of
a perforation with a complicating peribowel infection.
Extraintestinal manifestations — CD and ulcerative colitis share a number of extraintestinal

manifestations generally related to inflammatory disease activity (table 1) [5].
These manifestations, which tend to be more frequent with colonic involvement, include:
● Arthritis or arthropathy – Primarily involving large joints in approximately 20 percent of patients

without synovial destruction, arthritis is the most common extraintestinal manifestation. Central or
axial arthritis, such as sacroiliitis, or ankylosing spondylitis, may also occur. An undifferentiated
spondyloarthropathy or ankylosing spondylitis may be the presenting manifestation of CD. (See
"Clinical manifestations and diagnosis of arthritis associated with inflammatory bowel disease and
other gastrointestinal diseases".)
● Eye involvement – Eye manifestations in approximately 5 percent of patients include uveitis,

iritis, and episcleritis (picture 1A-B). (See "Dermatologic and ocular manifestations of
inflammatory bowel disease".)
● Skin disorders – Dermatologic manifestations occur in approximately 10 percent of patients and

include erythema nodosum and pyoderma gangrenosum (picture 2A-C). Rarely, vulvar
involvement of CD may manifest as swelling, pain, edema, erythema, and ulceration [8,9]. (See
"Vulvar lesions: Differential diagnosis based on morphology".)
● Primary sclerosing cholangitis – This typically presents in approximately 5 percent of patients

with an elevation in the serum alkaline phosphatase or gamma-glutamyl transpeptidase
concentration. (See "Primary sclerosing cholangitis in adults: Clinical manifestations and
diagnosis".)
● Secondary amyloidosis is very rare but may lead to renal failure and other organ system
involvement [10]. (See "Causes and diagnosis of AA amyloidosis and relation to rheumatic
diseases".)
● Venous and arterial thromboembolism resulting from hypercoagulability (table 2) [11-16]. (See
"Overview of medical management of high-risk, adult patients with moderate to severe Crohn
disease", section on 'Risk of venous thromboembolism'.)
● Renal stones – Calcium oxalate and uric acid kidney stones can result from steatorrhea and
diarrhea [17]. Uric acid stones can result from dehydration and metabolic acidosis. (See "Risk
factors for calcium stones in adults" and "Uric acid nephrolithiasis".)
● Bone loss and osteoporosis may result related to glucocorticoid use and impaired vitamin D
and calcium absorption [18,19]. (See "Metabolic bone disease in inflammatory bowel disease".)
● Vitamin B12 deficiency – A clinical picture of pernicious anemia can result from severe ileal
disease since vitamin B12 is absorbed in the distal 50 to 60 cm of ileum [20]. (See "Clinical
manifestations and diagnosis of vitamin B12 and folate deficiency".)
● Pulmonary involvement – Pulmonary manifestations of IBD include bronchiectasis, chronic

bronchitis, interstitial lung disease, bronchiolitis obliterans with organizing pneumonia (BOOP),
sarcoidosis, necrobiotic lung nodules, pulmonary infiltrates with eosinophilia (PIE) syndrome,
serositis, and pulmonary embolism. (See "Pulmonary complications of inflammatory bowel
disease", section on 'Primary respiratory involvement'.)
DIFFERENTIAL DIAGNOSIS — The extensive differential diagnosis associated with Crohn disease
(CD) varies with the site of involvement and the chronicity of the clinical presentation. In many
patients, the early symptoms of CD are mild and nonspecific. The differential diagnosis includes
irritable bowel syndrome (IBS), lactose intolerance, infectious colitis, and ulcerative colitis.
A consistent history (right lower quadrant pain, prolonged diarrhea, bleeding, fever, and family history
of inflammatory bowel disease [IBD]) and abnormal laboratory tests (anemia, iron deficiency) may
lead one to suspect CD.
Irritable bowel syndrome — Some gastrointestinal symptoms such as diarrhea and abdominal pain
are common to both IBS and IBD. The diagnostic evaluation may include ileocolonoscopy, imaging
studies, and/or stool markers. (See 'Stool markers' below and "Clinical manifestations and diagnosis
of irritable bowel syndrome in adults".)
Lactose intolerance — Intolerance to lactose-containing foods (primarily dairy products) is a

common problem and the diagnosis often initially confused with CD. Clinical symptoms of lactose
intolerance include diarrhea, abdominal pain, and flatulence after ingestion of milk or milk-containing
products. (See "Lactose intolerance: Clinical manifestations, diagnosis, and management".)
Infectious colitis — In patients presenting with diarrhea (especially with acute symptoms), infections
including Shigella, Salmonella, Campylobacter, Escherichia coli O157:H, Yersinia, parasites, and
amebiasis should be excluded. Clostridium difficile infection should be considered, particularly in
patients recently treated with antibiotics. In immunocompromised patients, cytomegalovirus (CMV)
infection can mimic CD.
One fresh stool sample for these agents should be obtained in most patients; in addition, tissue
endoscopic biopsies should be analyzed for CMV in immunocompromised patients.
In patients with primarily small bowel involvement, Yersinia can cause an acute ileitis indistinguishable
clinically from acute Crohn ileitis. Both tuberculosis and amebiasis can mimic CD of the ileum and
cecum. Sexually transmitted diseases (including Neisseria gonorrhoeae and Chlamydia trachomatis)
can cause rectal lesions and perianal fistulas that resemble CD [21]. (See "Giardiasis: Epidemiology,
clinical manifestations, and diagnosis" and "Causes of acute infectious diarrhea and other foodborne
illnesses in resource-rich settings".)
Ulcerative colitis — When CD involves the colon, it must be distinguished from ulcerative colitis
since both the medical and surgical therapies of these disorders can differ. Several findings suggest
CD:
● Involvement of the small bowel
● Sparing of the rectum
● Absence of gross bleeding
● Presence of perianal disease
● Focality of gross and microscopic lesions, the presence of granulomas, or the occurrence of
fistulas
In approximately 10 to 15 percent of patients with IBD, the distinction between CD and ulcerative
colitis cannot be made; such patients are referred to as having indeterminate colitis. Some patients
may initially be diagnosed with ulcerative colitis or CD but it evolves with time to the opposite
diagnosis. One report suggested that an evolution to a diagnosis of CD was more likely in patients
initially diagnosed with ulcerative colitis who presented with non-bloody diarrhea or weight loss [22].
Other disorders — Because of the segmental nature of CD, a variety of disorders can mimic the
clinical presentation. These include appendicitis, diverticulitis, diverticular colitis, ischemic colitis, and
a perforating or obstructing carcinoma. Lymphoma, chronic ischemia, endometriosis, and carcinoid
can all give a radiologic and clinical picture that is easily confused with CD of the small bowel.
DIAGNOSIS — The diagnosis of Crohn disease (CD) is usually established with endoscopic findings
or imaging studies in a patient with a compatible clinical history. Physical examination may be normal
or show nonspecific signs (pallor, weight loss) suggestive of CD. More specific findings include
perianal skin tags, sinus tracts, and abdominal tenderness. (See 'Clinical manifestations' above.)
Presenting symptoms frequently determine the order of subsequent testing. Colonoscopy is the most
appropriate initial test for patients presenting with predominant diarrhea, while imaging studies may be
more appropriate for those presenting with abdominal pain.
Laboratory studies — Initial blood studies for patients presenting with history and examination
suggesting CD should include:
● Complete blood count (CBC)
● Blood chemistry including electrolytes, renal function tests, liver enzymes, and blood glucose
● Erythrocyte sedimentation rate (ESR)
● C-reactive protein (CRP)
● Serum iron, vitamin D, and vitamin B12 levels
Routine laboratory tests may be normal or they may reveal anemia, iron deficiency, elevated white
blood cell count, B12 deficiency, and/or elevated erythrocyte sedimentation rate or CRP.
If diarrhea is present, a stool specimen should be sent for culture and examination for ova and
parasites, as well as C. difficile toxin in appropriate clinical settings.
Endoscopy
Colonoscopy — Colonoscopy with intubation of the terminal ileum is used to establish the
diagnosis of ileocolonic CD. Endoscopic features include focal ulcerations adjacent to areas of normal
appearing mucosa along with polypoid mucosal changes that give a cobblestone appearance (picture
3). (See "Endoscopic diagnosis of inflammatory bowel disease", section on 'Endoscopic findings in
Crohn disease'.)
Skip areas of involvement are typical with segments of normal appearing bowel interrupted by large
areas of disease. This pattern is different from the continuous involvement in ulcerative colitis.
Pseudopolyps (hypertrophied masses of mucous membrane resembling polyps), as seen in ulcerative
colitis, are also often present. Rectal sparing is common in CD as well. At the initial colonoscopy,
biopsies should be obtained from the right colon, left colon, and rectum even if endoscopically normal
in appearance to assess for microscopic inflammation.
The major findings on intestinal biopsy are focal ulcerations and acute and chronic inflammation.
These findings are usually confirmatory rather than diagnostic. The focality of the inflammation differs
from the diffuse pattern seen typically in ulcerative colitis.
Granulomas may be noted in up to 30 percent of patients with CD and are diagnostic of the disorder if
appropriate infections are excluded (picture 4). Thus, demonstration of a granuloma is not required for
establishing the diagnosis, nor does its presence confirm the diagnosis, since granulomas may be
seen with other disorders including Yersinia spp., Behçet syndrome, tuberculosis, and lymphoma.
Wireless capsule endoscopy — Wireless capsule endoscopy provides another means to

visualize the small bowel, and is being used increasingly for the evaluation of suspected and
established small bowel CD. It may detect suggestive lesions not visible by other small bowel studies
(picture 5). It should not be performed in patients with a suspected intestinal stricture since the
capsule may not be able to pass through the stricture, thus requiring surgical removal. It has the
advantage of providing visualization of the small bowel without exposing the patient to ionizing
radiation. Capsule endoscopy after a negative ileocolonoscopy and negative computed tomography
enterography (CTE) or small bowel follow-through (SBFT) has not been demonstrated to be a cost-
effective third test [10]. (See "Wireless video capsule endoscopy".)
Imaging studies — Imaging studies are most useful to evaluate the upper gastrointestinal tract and
allow documentation of the length and location of strictures in areas not accessible by colonoscopy.
Imaging has traditionally involved barium studies, such as barium enema or upper gastrointestinal
series with SBFT, though the use of CT and magnetic resonance enterography (MRE) is becoming
more standard of care in imaging in patients with CD.
Many examinations (eg, radiographs, barium enemas, CT scans, and fluoroscopy) expose patients to
ionizing radiation, which is associated with an increased risk of malignancy. In one study, the radiation
exposure from radiographic studies over a five-year period was assessed in 371 patients with CD [23].
The mean cumulative radiation exposure over five years was 14 milli-Sieverts (mSv), with a range of 0
to 303 mSv. The median cumulative radiation exposure was lower, at 3 mSv. While the majority of
patients had a cumulative exposure of less than 50 mSv (a cutoff used by some investigators to
identify patients at high risk of complications from radiation exposure), 27 patients (7 percent) had a
cumulative exposure of more than 50 mSv. The risks associated with radiation exposure have led
some investigators to advocate for the judicious use of studies that employ ionizing radiation,
especially in children and young adults, and prompted the development of contrast-enhanced
ultrasound to assess disease activity [23,24]. (See "Radiation-related risks of imaging".)
Colonic disease — Colonoscopy is the preferred study for evaluation of the lower bowel. Barium
enema is a less desirable alternative, but may be indicated when a complete colonoscopy cannot be
performed for technical reasons.
Air-contrast barium enema may not detect early subtle changes of CD, but may detect aphthous
lesions, and can document the extent of disease and the distribution and severity of early colonic
strictures. Sacculations may be seen in patients with chronic involvement (image 1A-B). It may also
reveal small perforations with fistula tracts.
Magnetic resonance imaging (MRI) can be used for the detection of perianal fistulas (image 2 and
image 3). The use of endoscopic ultrasound (EUS) to evaluate the perianal area can obviate the need
for surgical exploration. EUS can also be used in patients who cannot tolerate MRI. (See "Perianal
complications of Crohn disease" and "The role of imaging tests in the evaluation of anal abscesses
and fistulas", section on 'Magnetic resonance imaging' and "The role of imaging tests in the evaluation
of anal abscesses and fistulas", section on 'Endosonography'.)
Small bowel disease — Radiologic imaging is generally needed to evaluate patients with probable
small bowel disease. Several imaging modalities are available, including conventional upper
gastrointestinal series with SBFT, CT and CTE, enteroclysis, and MRI and MRE. We perform an MRE
as it has the advantage of avoiding radiation.
● Upper gastrointestinal series with SBFT involves ingestion of a barium solution with subsequent
radiologic imaging of the small intestine. Typical features of small bowel CD include narrowing of
the lumen with nodularity and ulceration, a "string" sign when luminal narrowing becomes more
advanced or with severe spasm, a cobblestone appearance, fistulas and abscess formation, and
separation of bowel loops, a manifestation of transmural inflammation with bowel wall thickening
(image 4A-G). Antral narrowing and segmental stricturing of the duodenum are suggestive of
gastroduodenal CD (image 5A-C).
● Standard CT (with barium solution) and CTE are useful for evaluating the small bowel, as well as
extraintestinal complications, such as intraabdominal abscesses [25]. CTE involves ingestion of a
neutral contrast agent to distend the small bowel, followed by CT imaging of the abdomen.
Neutral contrast allows for better evaluation of the wall of the small bowel, which is difficult to see
when standard barium solutions are used. CTE is a cost-effective alternative in patients with a
high pretest likelihood of CD [10]. CTE should be considered as the central study of the small
bowel if there is a clinical concern about an associated abscess (image 4G).
● MRI is another option for visualization of the small bowel and identification of extraluminal findings
and complications [26-28]. Increasing mural thickness, high mural signal intensity (possibly
reflecting edema), and a layered pattern of enhancement reflect histologic features of acute small
bowel inflammation on MRI [29]. MRE, like CTE, uses neutral contrast for better evaluation of the
wall of the small bowel and has high accuracy in the detection of inflammation associated with CD
[30,31].
In one study, 44 consecutive patients with ileal or ileocolonic CD underwent MRE, CTE, and
ileocolonoscopy for measurement of disease activity and detection of CD-related complications
[30]. Both CTE and MRE demonstrated similar accuracy for disease location, wall thickening and
enhancement, enlarged nodes, and involvement of perivisceral fat, but MRE demonstrated higher
accuracy in the detection of strictures (0.95 versus 0.91). In per-segment analysis, as compared
with CTE, MRE was significantly more accurate in the detection of ileal wall enhancement (0.88
versus 0.81). MRI also has the advantage of avoiding radiation exposure.
MRE may also be used to monitor response to therapy in patients with CD. A prospective study
included 48 patients with active CD and ulcers in at least one ileocolonic segment [32]. All
patients underwent ileocolonoscopy and MRE at baseline and 12 weeks after completing
treatment with glucocorticoids or an anti-tumor necrosis factor agent. MRE detected ulcer healing
with 90 percent accuracy and endoscopic remission with 83 percent accuracy when
ileocolonoscopy was used as a reference standard.
Multispectral optoacoustic tomography — Crohn disease activity can be assessed with

multispectral optoacoustic tomography (MSOT), a transabdominal imaging technique that detects
bowel wall inflammation by quantifying surrogate markers such as hemoglobin-dependent tissue
perfusion [33]. MSOT uses the excitation of short-pulsed laser light with near-infrared wavelengths to
induce the photoacoustic effect in targeted tissues, which results in sound waves generated by
thermoelastic expansion [34]. In a preliminary study using endoscopy as the reference standard in 44
patients with CD, MSOT parameters (ie, total hemoglobin, oxygenated hemoglobin and deoxygenated
hemoglobin) successfully distinguished active versus nonactive disease. Results were similar using
histology as the reference standard in 42 patients [33]. Although not yet available for use in practice
and further validation is needed, this technique holds promise for future clinical application.
Serologic markers — Disease markers to aid in the diagnosis of CD or indicate its severity have
been described; however, their accuracy and predictive value remain to be determined.
Antibody tests — A number of autoantibodies have been detected in patients with IBD. Antibody
tests have shown promise in distinguishing CD from ulcerative colitis and in predicting the disease
course of IBD in some reports [35-38]. However, prospective longitudinal studies are needed to clarify
whether these markers have real predictive value and should lead to aggressive therapy [39-41].
● Antineutrophil cytoplasmic antibodies (pANCA) and anti-Saccharomyces cerevisiae

antibodies (ASCA) – pANCA and ASCA have been proposed as a means for diagnosing IBD
and distinguishing CD from ulcerative colitis. One study included 582 adult patients with
established diagnoses (407 with CD, 147 with ulcerative colitis, and 28 with indeterminate colitis)
[42]. The sensitivity of the antibody tests alone or in combination was in the range of 40 to 60
percent, and the specificity was greater than 90 percent for distinguishing patients with IBD from
controls. Specificity was slightly less for distinguishing ulcerative colitis from CD (table 3). Similar
results have been described in several other reports [43,44].
The accuracy of these tests for categorizing patients with indeterminate colitis (the setting in
which they are most likely to be needed) is uncertain [42,45-47]. One of the largest prospective
studies to address this issue focused on 97 adults who underwent antibody testing and then were
followed clinically [47]. A diagnosis of ulcerative colitis or CD was established in 31 patients (32
percent). ASCA +/pANCA - correlated with CD in 8 of 10 patients, while ASCA -/pANCA +
correlated with UC in 7 of 11 patients.
ASCA were present in 50 percent of patients with celiac disease in one report, suggesting that
they may reflect a nonspecific immune response in the course of small bowel disease [48]. It has
also been described in association with cystic fibrosis [49] and intestinal tuberculosis [50].
● Anti-OmpC antibody – The anti-OmpC antibody has been identified as a potential serologic
marker of IBD [39,51,52]. The OmpC is an outer membrane porin, E. coli protein that is
immunoreactive to pANCA monoclonal antibodies [53]. Anti-OmpC antibodies were present in 46
percent of 303 adults with CD in one study [39].
● Additional antibody tests continue to be developed, including antibodies against laminaribioside,

chitobioside, or mannan [54], and CBir1 flagellin [55].
Although commercially available, the accuracy and predictive value of antibody tests and panels of
combinations of serologic tests for the diagnosis of IBD and the disease course and severity continue
to be elucidated [56,57]. Furthermore, the prevalence of these antibodies in patients with a variety of
inflammatory diseases affecting the gut has not been well studied. Thus, antibody tests should only be
used as an adjunct to conventional testing and clinical diagnosis and should not be used as screening
modalities.
C-reactive protein — Elevated levels of CRP have been observed in patients with IBD and are
generally higher in CD than in ulcerative colitis. CRP determination may have a role in distinguishing
between these diseases, as well as in differentiating patients with IBD from those with symptoms
caused by other disorders [58-62]. Levels of CRP are reported to correlate with CD activity [63-65].
Some studies have suggested that increased CRP levels predict the risk of relapse in patients with CD
[66,67], but discordant results have also been published [68].
It has been suggested that CRP may help in prediction of the outcome and risk of surgery and in
identification of patients who are likely to benefit most from specific treatments [69,70].
CRP levels are often used in clinical trials of therapy in IBD. One study, however, found that CRP
levels correlated with endoscopic but not clinical disease activity [71]. Furthermore, CRP levels varied
significantly based upon the presence of certain genetic polymorphisms.
CRP monitoring under therapy is useful in patients in whom it is elevated at baseline. Normalization of
CRP under therapy documents the efficacy of the anti-inflammatory therapy [72].
Genetic testing — Clinical genetic testing for the IBD 1 gene that encodes protein NOD2/CARD15 is
not presently recommended. NOD2/CARD15 mutations are present in the minority of patients with CD
and are not inherited in a strict Mendelian fashion [73]. The presence of NOD2 mutations is not
necessary for the diagnosis, and their absence does not rule out CD. (See "Immune and microbial
mechanisms in the pathogenesis of inflammatory bowel disease", section on 'Genetic susceptibility'.)
Stool markers — Although tests for fecal calprotectin or lactoferrin are not used routinely to diagnose
Crohn disease, they may help to differentiate patients with intestinal inflammation from patients with
functional bowel disease [74-77]. While calprotectin is used more commonly by some clinicians,
lactoferrin is an acceptable alternative [77,78]. (See "Approach to the adult with chronic diarrhea in
resource-rich settings", section on 'Fecal calprotectin'.)
In selected patients, those in whom it is difficult to distinguish Crohn disease from functional bowel
disease, for example, we measure fecal calprotectin instead of proceeding directly to ileocolonoscopy.
If fecal calprotectin is normal, a diagnosis of IBD is unlikely. If fecal calprotectin level is above the
reference range, we proceed with ileocolonoscopy and biopsy to confirm the diagnosis of IBD.
In a meta-analysis of eight studies including 1062 participants (565 patients with IBD [confirmed by
endoscopy], 259 patients with IBS and 238 healthy controls), which evaluated the performance of
fecal calprotectin in distinguishing between IBD and IBS, patients with a fecal calprotectin level of <40
microg/g had a 1 percent chance or less of having IBD [77]. In a separate analysis of two studies in
the same systematic review (541 participants [275 patients with IBD, 168 patients with IBS and 98
healthy controls]), patients with a fecal lactoferrin level of <10microg/g had a 2 percent chance or less
of having IBD. (See "Clinical manifestations and diagnosis of irritable bowel syndrome in adults",
section on 'Laboratory testing'.)
In an earlier meta-analysis that included six studies with 670 adult patients, the presence of fecal
calprotectin was 93 percent sensitive and 96 percent specific for identifying patients with IBD [79],
using ileocolonoscopy with histology as the reference standard.
We also measure fecal calprotectin when evaluating a patient with established Crohn disease who
had achieved clinical remission but who now presents with symptoms of a disease flare (eg,
abdominal pain, diarrhea). In this setting, some clinicians may initiate treatment based on clinical
evaluation, but we obtain objective testing (eg, stool markers, laboratory studies) prior to initiating
immunosuppressive therapy.
The use of fecal calprotectin for monitoring disease activity in patients with IBD is discussed
separately. (See "Management of Crohn disease after surgical resection", section on 'Postoperative
monitoring' and "Management of mild to moderate ulcerative colitis in adults", section on 'Laboratory
monitoring'.)
CANCER RISK — Studies conflict regarding the risk of colon cancer in patients with longstanding
Crohn colitis [80-83]. This is discussed in detail elsewhere. (See "Surveillance and management of
dysplasia in patients with inflammatory bowel disease", section on 'Crohn disease'.)
Increases in incidence of squamous cell carcinoma of the anus and skin, duodenal neoplasia,
testicular cancer, leukemia, and miscellaneous other cancers have all been reported in Crohn disease
(CD), although the strength of these associations is unclear [84]. In a retrospective population-based
cohort study that included 3348 patients with inflammatory bowel disease (IBD) and no prior cancer
history, both the overall cancer risk and the risk of colon cancer was not increased [85]. However,
patients with IBD had a higher risk of hematologic malignancies (standardized incidence ratios [SIR]
CD, 14, 95% CI 6.7-25.9; SIR ulcerative colitis 2.5 95% CI 1.2-4.6).
Patients receiving thiopurines for inflammatory bowel disease have an increased risk of developing
lymphoproliferative disorders [86]. Analyses of lymphoma risk in patients receiving biologic agents
directed against tumor necrosis factor (TNF)-alpha are confounded by concomitant use of
immunosuppressive agents in most of these patients. It is unclear whether exposure to both an

immunomodulator and an anti-TNF agent increases the risk of non-Hodgkin lymphoma relative to
those treated with immunomodulators alone [87,88] (see "Overview of azathioprine and
6-mercaptopurine use in inflammatory bowel disease"). However, one type of lymphoma, the
hepatosplenic T-cell lymphoma, seems to occur mainly in young male patients receiving a
combination of anti-TNF and azathioprine [87].
PROGNOSIS — The typical course in patients with Crohn disease (CD) involving the small and/or
large intestine is one of intermittent exacerbation of symptoms followed by periods of remission.
Approximately 10 to 20 percent of patients experience a prolonged remission after initial presentation

[89,90]. Another study found that 53 percent of patients developed stricturing or penetrating disease at
10 years follow-up [90]. Predictors of a severe course include age less than 40, the presence of
perianal or rectal disease, smoking, low education level, and initial requirement for glucocorticoids
[91,92].
In a study of 306 patients (45 percent with ileal disease, 32 percent with colonic disease, and 19
percent with ileocolonic disease), 81 percent had nonstricturing/nonpenetrating disease at baseline
[93]. The cumulative risk of developing either complication at 90 days was 19 percent, at 1 year was
22 percent, at 5 years was 34 percent, and at 20 years was 51 percent. Factors associated with
complications included disease extent at baseline. Patients with ileal disease had a ninefold increased
risk compared with those with colonic disease, and patients with ileocolonic disease had a sixfold
increased risk. In addition, use of mesalamine or sulfasalazine in the first 90 days increased the risk of
complications twofold. Finally, perianal disease increased the risk of complications, though the results
were of borderline significance (hazard ratio 1.69, 95% CI 0.99 to 2.86).
The following observations were made in a guideline published by the American College of
Gastroenterology [75]:
● Up to 80 percent of patients require hospitalization during the course of their disease.
● For most patients, symptoms are chronic and intermittent, while smaller subsets of patients have
either a continuous course of active disease or experience prolonged remission.
● The five-year rate of symptomatic, post-operative recurrence is approximately 50 percent.
Many patients with CD ultimately require surgical intervention with intestinal resection because of
intractability of symptoms, obstruction, or perforation. Some patients tend to follow a pattern of either
recurrent obstruction or recurrent perforations; the latter group has been reported to have a more
aggressive form of the disease, leading to earlier postoperative recurrence and the need for more
surgery. Genetic studies have suggested that there may be a molecular basis for more aggressive
disease [94,95]. However, clinically distinct disease patterns have not been observed in all studies
[96,97]. (See "Surgical management of ulcerative colitis".)
Mortality — Studies addressing whether overall life expectancy is decreased in patients with CD have
produced disparate results, reflecting the heterogeneity of the disease, biases related to specific study
populations, and variable lengths of follow-up [98-107].
Estimates of the standardized mortality ratio (an approximation of the risk of death compared to the
general population) from population-based studies range from no increased risk to a fivefold increased
risk of death [105,106,108-110]. This range reflects a varying spectrum of disease severity in different
populations. An interpretation of these data is that CD is associated with only a modest decrease in
overall life expectancy.
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from

selected countries and regions around the world are provided separately. (See "Society guideline
links: Crohn disease in adults".)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The
Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at
the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have
about a given condition. These articles are best for patients who want a general overview and who
prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more
sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and
are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or
e-mail these topics to your patients. (You can also locate patient education articles on a variety of
subjects by searching on "patient info" and the keyword(s) of interest.)
● Basics topics (see "Patient education: Crohn disease in adults (The Basics)")
● Beyond the Basics topics (see "Patient education: Crohn disease (Beyond the Basics)")
SUMMARY AND RECOMMENDATIONS
● Crohn disease (CD) is a disorder of uncertain etiology characterized by transmural inflammation

of the gastrointestinal tract. CD may involve the entire gastrointestinal tract from the mouth to the
perianal area. (See 'Introduction' above.)
● The clinical manifestations of CD are variable, and patients can have symptoms for many years
prior to diagnosis. Prolonged diarrhea with abdominal pain, with or without gross bleeding,
fatigue, and weight loss are the hallmarks of CD. (See 'Clinical manifestations' above.)
● Patients can present with symptoms secondary to the transmural involvement of the bowel,
including fistulas, phlegmon, abscess, perianal disease, and/or malabsorption. Extraintestinal
manifestations, such as arthritis or arthropathy, eye and skin disorders, biliary tract involvement,
and kidney stones, may occur and tend to be more frequent with colonic involvement. (See
'Clinical manifestations' above.)
● The extensive differential diagnosis associated with CD varies with the site of involvement and
the chronicity of the clinical presentation. (See 'Differential diagnosis' above.)
● The diagnosis of CD is established with endoscopic and imaging studies of the bowel in a patient
with a compatible clinical history. Colonoscopy with intubation of the terminal ileum is used to
establish the diagnosis of ileocolonic CD. We perform a magnetic resonance enterography as the
initial test to evaluate the small intestine. Wireless capsule endoscopy may also be useful for
detecting small bowel involvement. (See 'Diagnosis' above.)
● Although several serologic markers for CD are commercially available, the accuracy and
predictive value of these markers for the diagnosis of CD remain to be elucidated. (See 'Serologic
markers' above.)
● The typical course in patients with CD involving the small and/or large intestine is one of
intermittent exacerbations of symptoms followed by periods of remission. (See 'Prognosis'
above.)
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Topic 4070 Version 36.0
GRAPHICS
Extraintestinal manifestations of inflammatory bowel disease
Common extraintestinal manifestations
Musculoskeletal
Arthritis - Colitic type, ankylosing spondylitis, isolated joint involvement such as sacroiliitis
Hypertrophic osteoarthropathy - Clubbing, periostitis, metastatic Crohn disease
Miscellaneous - Osteoporosis, aseptic necrosis, polymyositis, osteomalacia
Skin and mouth
Reactive lesions - Erythema nodosum, pyoderma gangrenosum, aphthous ulcers, vesiculopustular

eruption, cutaneous vasculitis, neutrophilic dermatosis, metastatic Crohn disease, epidermolysis bullosa
acquisita
Specific lesions - Fissures and fistulas, oral Crohn disease, drug rashes
Nutritional deficiency - Acrodermatitis enteropathica (zinc), purpura (vitamins C and K), glossitis
(vitamin B), hair loss and brittle nail (protein)
Associated diseases - Vitiligo, psoriasis, amyloidosis, epidermolysis bullosa acquisita
Hepatobiliary
Specific complications - PSC and bile duct carcinoma, small duct PSC, cholelithiasis
Associated inflammation - Autoimmune chronic active hepatitis, pericholangitis, portal fibrosis and
cirrhosis, granuloma in Crohn disease
Metabolic - Fatty liver, gallstones associated with ileal Crohn disease
Ocular
Uveitis iritis, episcleritis, scleromalacia, corneal ulcers, retinal vascular disease, retrobulbar neuritis,
Crohn keratopathy
Metabolic
Growth retardation in children and adolescents, delayed sexual maturation
Less common extraintestinal manifestations
Blood and vascular
Anemia due to iron, folate, or B12 deficiency or autoimmune hemolytic anemia, anemia of chronic
disease, thrombocytopenic purpura; leukocytosis and thrombocytosis; thrombophlebitis and
thromboembolism, arteritis and arterial occlusion, polyarteritis nodosa, Takayasu arteritis, cutaneous
vasculitis, anticardiolipin antibody, hyposplenism.
Renal and genitourinary tract
Urinary calculi (oxalate stones in ileal disease), local extension of Crohn disease involving ureter or
bladder, amyloidosis, drug-related nephrotoxicity.
Renal tubular damage with increased urinary excretion of various enzymes (eg, beta N-acetyl-D-
glucosaminidase).
Neurologic
Up to 3 percent of patients may have non-iatrogenic neurologic involvement, including peripheral

neuropathy, myelopathy, vestibular dysfunction, pseudotumor cerebri, myasthenia gravis, and
cerebrovascular disorders. Incidence equal in ulcerative colitis and Crohn disease. These disorders
usually appear five to six years after the onset of inflammatory bowel disease and are frequently
associated with other extraintestinal manifestations.
Airway and parenchymal lung disease
Pulmonary fibrosis, vasculitis, bronchitis, necrobiotic nodules, acute laryngotracheitis, interstitial lung
disease, sarcoidosis. Abnormal pulmonary function tests without clinical symptoms are common (up to
50 percent of cases).
Cardiac
Pericarditis, myocarditis, endocarditis, and heart block: more common in ulcerative colitis than in Crohn
disease; cardiomyopathy, cardiac failure due to anti-TNF therapy.
Pericarditis may also occur from sulfasalazine/5-aminosalicylates.
Pancreas
Acute pancreatitis: more common in Crohn disease than in ulcerative colitis. Risk factors include
6-mercaptopurine and 5-aminosalicylate therapy, duodenal Crohn disease.
Autoimmune
Drug-induced lupus and autoimmune diseases secondary to anti-TNF-alpha therapy.
Positive ANA, anti-double-stranded DNA, cutaneous and systemic manifestations of lupus.
PSC: primary sclerosing cholangitis; ANA: antinuclear antibody; TNF: tumor necrosis factor.
Modified from: Das KM. Relationship of extraintestinal involvements in inflammatory bowel disease: New insights
into autoimmune pathogenesis. Dig Dis Sci 1999; 44:1.
Graphic 81867 Version 10.0
Anterior uveitis in a patient with inflammatory bowel

disease
Anterior uveitis in a patient with inflammatory bowel disease is characterized by

injection of the conjunctiva and opacity in the anterior chamber.
Courtesy of the American Gastroenterological Association©. This slide cannot be

downloaded but may be purchased as part of a set from the AGA through Milner-
Fenwick, Inc at 1-800-432-8433.
Episcleritis
Patient with episcleritis associated with inflammatory bowel disease showing the
characteristic injection of the ciliary vessels.

Fenwick, Inc at 1-800-432-8433.
Erythema nodosum
Patient with inflammatory bowel disease with red nodular areas on the shins
which are characteristic of erythema nodosum.

Fenwick, Inc at 1-800-432-8433.
Pyoderma gangrenosum
Early lesion in pyoderma gangrenosum presenting as a pustular and violaceous

plaque with incipient breakdown.
Courtesy of Cynthia Magro, MD.
Pyoderma gangrenosum
Multiple active and healing lesions of pyoderma gangrenosum with cribriform

scarring in patient with inflammatory bowel disease.
Courtesy of Samuel Moschella, MD.
Hypercoagulability in inflammatory bowel disease
Clinical characteristics
Unusual sites
Associated with active disease and better when disease controlled
Associated with use of steroids (possibly indicating active disease)
Recurrent
Serious
Younger age
Abnormalities described
Abnormal fibrinolysis
Abnormal platelet aggregation
Activated protein C increased
Circulating immune complexes
Decreased antithrombin III
Factor V Leiden mutation
Increased cytokines (interleukin-6, thrombopoietin)
Increased factors V and VIII
Increased plasminogen activator inhibitor
Increased sedimentation rate, fibrinogen
Lupus anticoagulant
Thrombocytosis and leukocytosis (uniformly present in most studies)
Endoscopic findings in Crohn disease
The dominant endoscopic feature in Crohn disease is the presence of

ulcerations. Endoscopic findings in Crohn disease include: aphthous ulcers,
which are the earliest lesions seen in Crohn disease (panel A); large ulcers
interspersed with normal mucosa, which are typical for the segmental
distribution of Crohn disease (panel B); a cobblestone appearance that is
characterized by nodular thickening, with linear or serpiginous ulcers (panel C);
and strictures due to fibrosis (panel D).
Courtesy of Paul Rutgeerts, MD, PhD, FRCP.
Typical granuloma of Crohn disease
Light micrographs showing a granulomatous lesion that is diagnostic of Crohn

disease. Low and high power views show a central giant cell surrounded by
epitheliod cells and rimmed by lymphocytes.

Fenwick, Inc at 1-800-432-8433.
Crohn disease
Small bowel ulceration as seen during capsule endoscopy.
Courtesy of Given Imaging, Inc.
Aphthoid ulcers in Crohn disease
Double contrast barium enema demonstrates small aphthoid ulcers in a patient

with early Crohn disease of the colon (arrows). Barium has collected in the
superficial erosions, which are surrounded by edematous mucosa. These small
erosions, less typically seen in ulcerative colitis, are the precursors of larger
discrete ulcers, fistulae, and sinus tracts.
Courtesy of Jonathan Kruskal, MD, PhD.
Chronic Crohn colitis
Barium enema demonstrates sacculations along the medial border of the

ascending colon (arrows) produced by scarring and fibrosis in a patient with
Crohn disease.
Perianal fistulas in Crohn disease
T2-weighted axial magnetic resonance imaging (MRI) study showing perianal

fistulas (arrows) in a patient with Crohn disease.
Courtesy of Jonathan Kruskal, MD.
Perianal fistulas in Crohn disease
T2-weighted coronal magnetic resonance imaging (MRI) study showing a

perianal fistula (arrows) in a patient with Crohn disease.
Courtesy of Jonathan Kruskal, MD.
Crohn disease
Small bowel follow through examination demonstrates nodular filling defects

arising on thickened folds in the terminal ileum (arrows). These features are
characteristic of Crohn disease.
Crohn colitis
Double contrast barium enema in a patient with Crohn disease shows extensive
ulceration of the wall of the colon associated with mucosal thickening and
inflammation.
String sign in Crohn disease
Small bowel follow through study shows marked narrowing, irregularity and
ulceration in the distal ileum (arrows) in a patient with Crohn disease.
Cobblestone appearance in Crohn disease
Small bowel follow through study demonstrates diffuse thickening of the small
bowel mucosa in a patient with Crohn disease. The cobblestone appearance is
produced by barium being dispersed between the edematous inflamed mucosa.
Courtesy of Norman Joffe, MD.
Ileocecal fistulae in Crohn disease
Small bowel follow through examination demonstrates nodular thickening of the

terminal ileal mucosal folds in a patient with Crohn disease (arrow). Several
fistulae extend from the terminal ileum to the adjacent cecum (arrowheads).
Crohn disease with abscess and fistulae
Small bowel follow through study demonstrates an abscess cavity (arrow) with
fistulae connecting the cavity to the adjacent small bowel (arrowheads). Note
the marked thickening of the inflamed mucosal folds (dashed arrows).
Right lower quadrant abscess in Crohn disease
Single axial CT scan of the lower abdomen demonstrates an abscess

(arrowheads) extending from the markedly thickened and inflamed terminal
ileum (arrow). The presence of contrast material within the abscess confirms a
communication with the adjacent ileum.
Courtesy of Norman Joffe, MD.
Crohn disease of the upper gastrointestinal tract
Upper gastrointestinal series in a patient with gastroduodenal Crohn disease

shows antral narrowing (small arrow) and two duodenal strictures (large
arrows).

Fenwick, Inc at 1-800-432-8433.
Crohn disease of the stomach
This upper gastrointestinal series, performed in a young man with known Crohn
disease of the terminal ileum, shows numerous rounded filling defects in the
stomach produced by edematous mucosa. In some of these areas, small central
collections of barium are demonstrated (arrow), resulting from superficial
erosions. These features are suggestive of Crohn disease, but may also be seen
in patients with peptic ulcer disease and in those with viral gastritis.
Normal upper GI series
Normal air-contrast upper GI study showing normal gastric folds and small
intestinal anatomy, and no masses.
GI: gastrointestinal.
Courtesy of Paul C Schroy III, MD.
Test characteristics of anti-saccaromyces cervisiae antibodies (ASCA) and

perinuclear antineutrophil cytoplasmic antibodies (pANCA) in the diagnosis
of inflammatory bowel disease (IBD)
Antibody Sensitivity, percent Specificity, percent PPV, percent
Sensitivity, specificity, and positive predictive value (PPV) in patients with IBD compared to
non-IBD controls
ASCA + 60 91 88
pANCA + 50 95 69
ASCA +/pANCA - 56 94 91
pANCA +/ASCA - 44 97 78
Sensitivity, specificity, and positive predictive value in differentiating ulcerative colitis (UC) from
Crohn disease (CD) in patients with IBD*
ASCA +/CD + 60 86 92
pANCA +/UC + 50 94 76
ASCA +/pANCA -/CD + 56 92 95
pANCA +/ASCA -/UC + 44 98 88
* Where CD + or UC +, the control group for comparison is the other type of IBD.
Data from: Peeters M, Joosens S, Vermeire S, et al. Diagnostic value of anti-Saccharomyces cerevisiae and
antineutrophil cytoplasmic autoantibodies in inflammatory bowel disease. Am J Gastroenterol 2001; 96:730.

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Clinical manifestations, diagnosis and prognosis of Crohn disease in adults - UpToDate Page 1 of 46

Official reprint from UpToDate®

Clinical manifestations, diagnosis and prognosis of Crohn disease in adults

Authors: Mark A Peppercorn, MD, Sunanda V Kane, MD, MSPH

INTRODUCTION — Crohn disease (CD) is a disorder of uncertain etiology that is characterized by

● Approximately one-third of patients have perianal disease.

● Approximately 5 to 15 percent have predominant involvement of the mouth or gastroduodenal

● Bile salt malabsorption due to an inflamed or resected terminal ileum

● Steatorrhea related to loss of bile salts

● Small intestinal bacterial overgrowth

● Overlapping irritable bowel syndrome

● Enterocutaneous fistulas causing bypass of portions of absorptive surface area

● Enteroenteric fistulas may be asymptomatic or present as a palpable mass

Other gastrointestinal involvement — The clinical manifestations of other sites of gastrointestinal

● Esophageal involvement may present with odynophagia and dysphagia.

Extraintestinal manifestations — CD and ulcerative colitis share a number of extraintestinal

● Arthritis or arthropathy – Primarily involving large joints in approximately 20 percent of patients

● Eye involvement – Eye manifestations in approximately 5 percent of patients include uveitis,

● Skin disorders – Dermatologic manifestations occur in approximately 10 percent of patients and

● Primary sclerosing cholangitis – This typically presents in approximately 5 percent of patients

● Pulmonary involvement – Pulmonary manifestations of IBD include bronchiectasis, chronic

Lactose intolerance — Intolerance to lactose-containing foods (primarily dairy products) is a

● Involvement of the small bowel

● Sparing of the rectum

● Absence of gross bleeding

● Presence of perianal disease

● Complete blood count (CBC)

● Erythrocyte sedimentation rate (ESR)

● C-reactive protein (CRP)

● Serum iron, vitamin D, and vitamin B12 levels

Wireless capsule endoscopy — Wireless capsule endoscopy provides another means to

Multispectral optoacoustic tomography — Crohn disease activity can be assessed with

● Antineutrophil cytoplasmic antibodies (pANCA) and anti-Saccharomyces cerevisiae

● Additional antibody tests continue to be developed, including antibodies against laminaribioside,

immunosuppressive agents in most of these patients. It is unclear whether exposure to both an

Approximately 10 to 20 percent of patients experience a prolonged remission after initial presentation

● Up to 80 percent of patients require hospitalization during the course of their disease.

● The five-year rate of symptomatic, post-operative recurrence is approximately 50 percent.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from

SUMMARY AND RECOMMENDATIONS

● Crohn disease (CD) is a disorder of uncertain etiology characterized by transmural inflammation

Use of UpToDate is subject to the Subscription and License Agreement.

Topic 4070 Version 36.0

Extraintestinal manifestations of inflammatory bowel disease

Common extraintestinal manifestations

Hypertrophic osteoarthropathy - Clubbing, periostitis, metastatic Crohn disease

Miscellaneous - Osteoporosis, aseptic necrosis, polymyositis, osteomalacia

Skin and mouth

Reactive lesions - Erythema nodosum, pyoderma gangrenosum, aphthous ulcers, vesiculopustular

Associated diseases - Vitiligo, psoriasis, amyloidosis, epidermolysis bullosa acquisita

Metabolic - Fatty liver, gallstones associated with ileal Crohn disease

Growth retardation in children and adolescents, delayed sexual maturation

Less common extraintestinal manifestations

Blood and vascular

Renal and genitourinary tract

Up to 3 percent of patients may have non-iatrogenic neurologic involvement, including peripheral

Airway and parenchymal lung disease

Pericarditis may also occur from sulfasalazine/5-aminosalicylates.