Crohn Disease Clinical Presentation

  • Author: Priya Rangasamy, MD; Chief Editor: Julian Katz, MD   more...
 
Updated: Jun 16, 2011
 

History

Patients with suspected Crohn disease should be evaluated initially by their primary care team. Symptoms should be elicited in detail. A medical history, detailed review of systems, and family history should be obtained.

In children, growth parameters should be documented; growth failure may precede GI symptoms by years. The etiology of growth failure is multifactorial, with nutritional, hormonal, and disease-related factors all contributing. Any child or adolescent with persistent alterations in growth or delayed puberty should undergo appropriate diagnostic evaluation for Crohn disease.

General manifestations

Low-grade fever, prolonged diarrhea with abdominal pain, weight loss, and generalized fatigability are usually reported. The patient may develop crampy or steady right lower quadrant or periumbilical pain. The pain precedes and may be partially relieved by defecation. Diarrhea is usually not grossly bloody and is often intermittent. If the colon is involved, patients may report diffuse abdominal pain accompanied by mucus, blood, and pus in the stool.[2, 3, 6, 14, 15]

Patients with Crohn disease may also present with complaints that are suggestive of intestinal obstruction. Initially, the obstruction is secondary to inflammatory edema and spasm of the bowel and manifests as postprandial bloating, cramping pains, and loud borborygmi. Once the bowel lumen becomes chronically narrowed, patients may complain of constipation and obstipation. Complete obstruction may sometimes be caused by impaction of undigested foods.

Cologastric fistulae may manifest as feculent vomiting, enterovesical fistulae as recurrent urinary tract infections and pneumaturia, enterovaginal fistulae as feculent vaginal discharge, and enterocutaneous fistulae as feculent soiling of the skin. Development of fistulae into the mesentery may result in intra-abdominal or retroperitoneal abscess formation.

Location and extent

The location and extent of the disease primarily determines the patient's clinical presentation. Although any area of the GI system may be affected in patients with Crohn disease, the most common site of the chronic inflammatory process is the ileocecal region, followed by the colon, the small intestine alone, the stomach (rarely), and the mouth. The esophagus is very rarely involved.[2] The terminal ileum is involved in 50-70% of children. More than half of these patients also have inflammation in various segments of the colon, usually the ascending colon. Gastric inflammation, duodenal inflammation, or both may be observed in as many as 30-40% of children with Crohn disease. The primary pancreatic manifestation is pancreatitis.

Crohn disease of the small intestine usually presents with evidence of malabsorption, including diarrhea, abdominal pain, weight loss, and anorexia. Initially, these symptoms may be quite subtle. However, patients with upper GI Crohn disease may experience nausea, vomiting, and abdominal pain as dominating presenting symptoms.

Colonic Crohn disease may be clinically indistinguishable from ulcerative colitis, with symptoms of bloody mucopurulent diarrhea, cramping abdominal pain, and urgency to defecate.

Nearly 30% of patients with either large bowel or small bowel disease develop perianal complications. Perianal complications may precede the development of intestinal symptoms and manifest as simple skin tags, anal fissures, perianal fistulae, or abscesses.[2] Symptoms of painful defecation, bright red rectal bleeding, and perirectal pain, erythema, or discharge may signal perianal disease and may occur without symptomatic involvement in any other area of the GI tract.

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Physical Examination

The physical examination should focus on the patient's temperature, weight, nutritional status, presence of abdominal tenderness or a mass, perianal and rectal examination findings, and extraintestinal manifestations.

Vital signs are usually normal, although tachycardia may be present in anemic patients. Chronic intermittent fever is a common presenting sign.

Abdominal findings may vary from normal to those of an acute abdomen. Diffuse abdominal tenderness is often present. Fullness or a discrete mass may be appreciated, typically in the right lower quadrant of the abdomen (which is typical for ileal involvement), or a mass can sometimes be felt secondary to thickened or matted loops of inflamed bowel.

The perineum should be inspected in all patients who present with signs and symptoms of Crohn disease, because abnormalities detectable in this region substantially increase the clinical suspicion of IBD. Inspection of the perianal region can reveal skin tags, fistulae, abscesses, and scarring. A rectal examination can help determine sphincter tone and help detect gross abnormalities of the rectal mucosa or the presence of hematochezia.

In addition to local complications, a variety of extraintestinal manifestations may be associated with Crohn disease. The usual sites are the skin, joints, mouth, eyes, liver, and bile ducts.[16] Examination of the skin and oral mucosa may show mucocutaneous ulcers, erythema nodosum, and pyoderma gangrenosum. Skin examination may also reveal pallor in patients with anemia or jaundice in those with concomitant liver disease. The most common extraintestinal manifestations of Crohn disease are arthritis and arthralgia. The large joints (eg, hips, knees, ankles) are typically involved.[12] Eye examination may reveal episcleritis. For the diagnosis of uveitis, a slit lamp examination by an experienced physician is necessary.

Careful assessment of growth and development is an important part of evaluating pediatric patients. Growth abnormalities may be detected by evaluating several parameters: height and weight, percentage height and weight for the patient's age and percentage weight for the patient's height, growth velocity, body composition on anthropometry, and skeletal bone age. The most sensitive indicator of growth abnormalities is a decrease in growth velocity, which may be observed before the major percentile lines on standard growth curves are crossed. Decrease in height velocity before the onset of intestinal symptoms can be observed in as many as 46% of patients with Tanner stage 1 or 2. Height at maturity is often compromised. Pubertal delay may precede the onset of intestinal symptoms, and accurate Tanner staging should be a part of routine physical examination.

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Intestinal Manifestations

The major intestinal complications of Crohn disease are due to the transmural nature of the disease. This leads to the formation of abscesses, fistulae, sinus tracts (incomplete fistulae ending in a "cul de sac"), strictures, and adhesions, which may also contribute to obstruction.

Frank perforation is one of the most serious complications of Crohn disease. Perforation typically occurs into other segments of bowel, leading to fistulae, or to areas such as the retroperitoneum, resulting in abscess formation. The presenting features of frank perforation are those of classic peritonitis, although high-dose corticosteroid therapy may mask these features.

Fistula and abscess formation in Crohn disease is due to transmural bowel perforation. Perianal and perirectal fistulization are most common. Proper evaluation of perianal disease requires a combination of 2 of the following: pelvic MRI, examination under anesthesia, or endoscopic ultrasonography. Other complications of fistulizing disease include enterovesical and enterocutaneous fistulas.

Colonic malignancy is a clinically significant complication of Crohn disease in patients with pancolitis beginning in childhood. Although the risk of malignancy in Crohn disease is not as high as that in ulcerative colitis, the risk of adenocarcinoma of the colon in Crohn colitis is 4-20 times that of the general population. Small intestinal carcinoma is 50-100 times more likely to develop in patients with small intestinal Crohn disease but is still rare. The risk for children with an onset of disease in the first decade is unknown, but children who develop colitis when younger than 10 years should undergo colonoscopic screening during adolescence. Epithelial dysplasia generally precedes carcinoma; therefore, yearly surveillance colonoscopy is recommended for patients with this condition, who are at high risk.

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Extraintestinal Manifestations

The extraintestinal manifestations of Crohn disease may carry prognostic importance.

Musculoskeletal diseases

The most common extraintestinal manifestation in children and adolescents is arthritis (7-25% of pediatric patients). The arthritis is usually seronegative, transient, nondeforming, and asymmetric in distribution and involves the large joints of the lower extremities. In adults, the arthritis occurs when the disease is active and can affect large and small joints. In children, the arthritis may occur years before any GI symptoms develop, and may persist after surgical or medical remission of the disease.

Other musculoskeletal manifestations include the following:

  • Arthralgia
  • Ankylosing spondylitis
  • Psoriatic arthritis
  • Reactive arthritis
  • Sacroiliitis

Skin diseases

The most common skin manifestation of Crohn disease is erythema nodosum. Erythema nodosum is more common in Crohn disease than in ulcerative colitis and usually follows the course of the disease. Erythema nodosum affects 3% of pediatric patients with Crohn disease, which is less frequent than in adults. Approximately 75% of patients with erythema nodosum ultimately develop arthritis. The lesions of erythema nodosum are raised, red, tender nodules that appear primarily on the anterior surfaces of the lower leg. Pyoderma gangrenosum is uncommon in Crohn disease. Pyoderma gangrenosum is often an indolent chronic ulcer, which may occur even when the disease is in remission. Therefore, medical therapy for the underlying bowel disease is not always successful.

Other dermatologic manifestations include the following:

  • Sweet syndrome
  • Orofacial granulomatosis
  • Angular and aphthous stomatitis
  • Acrodermatitis enteropathica
  • Alopecia
  • Metastatic Crohn disease
  • Crohn disease of the vulva and penis

Oral lesions

Aphthous ulceration in the mouth is the most common oral manifestation of Crohn disease. This ulceration is commonly associated with skin and joint lesions. Oral lesions appear to parallel intestinal disease in most cases, but they may also occur before any GI symptoms occur.

Ophthalmologic diseases

Ophthalmologic manifestations of Crohn disease (primarily episcleritis and anterior uveitis) most frequently occur when the disease is active. The rate is 4% in the adult population but is lower in children and adolescents. The uveitis is usually symptomatic, causing pain or decreased visual acuity. Increased intraocular pressure and cataracts may be observed in children who receive corticosteroid therapy. Conjunctivitis also occurs. All patients with Crohn disease require ophthalmologic examination at regular intervals.

Urologic diseases

Urologic manifestations of Crohn disease include nephrolithiasis, hydronephrosis, and enterovesical fistulae. Nephrolithiasis occurs in less than 5% of children with Crohn disease. Nephrolithiasis is usually the result of fat malabsorption that occurs with small bowel Crohn disease. Dietary calcium binds to malabsorbed fatty acids in the colonic lumen; therefore, free oxalate is absorbed. The absorption of free oxalate results in hyperoxaluria and oxalate stones. In patients with an ileostomy, increased fluid and electrolyte losses may lead to concentrated acidic urine and the formation of uric acid stones. External compression of the ureter by an inflammatory mass or abscess may lead to hydronephrosis. Enterovesical fistulae may present with recurrent urinary tract infections or pneumaturia.

Hepatobiliary diseases

Hepatobiliary disease is among the most common extraintestinal manifestations of Crohn disease and its therapies. Abnormal serum aminotransferases are common during the course of Crohn disease in children. Most aminotransferase elevations are transient and appear to relate to medications or disease activity. Persistent aminotransferase elevations (>6 mo) should be investigated because the likelihood of serious liver disease is increased. Both intrahepatic and extrahepatic manifestations of liver disease occur in children with Crohn disease. Intrahepatic manifestations include chronic active hepatitis, granulomatous hepatitis, amyloidosis, fatty liver, and pericholangitis. Extrahepatic manifestations include cholelithiasis and obstruction. Cholelithiasis is more frequent in patients with IBD than in the general population, probably because of bile salt pool alteration from malabsorption. Chronic active hepatitis and sclerosing cholangitis develops in less than 1% of children with Crohndisease.Sometimes, ahepatic abscess manifests as fever of unexplained origin.

Other hepatobiliary manifestations include the following:

  • Cirrhosis
  • Portal vein thrombosis
  • Cholangiocarcinoma

Thromboembolic diseases

Thromboembolic disease is considered the result of a hypercoagulable state that parallels disease activity and is manifested by thrombocytosis, elevated plasma fibrinogen, factor V, factor VIII, and decreased plasma antithrombin III. This may lead to deep vein thrombosis, pulmonary emboli, and neurovascular disease. Compared with controls, patients with IBD have a 3-fold higher risk of thromboembolism. These patients have frequent exposure to classic thrombosis risk factors, including immobility, surgery, steroid therapy, and the presence of central venous catheters. Other factors that may play a role include smoking, antiphospholipid antibody syndrome, and hyperhomocystinemia.

Additional extraintestinal diseases

Bone metabolic disorders include osteopenia and osteoporosis.

Hematologic manifestations include iron deficiency anemia, vitamin B-12 deficiency anemia, folate deficiency anemia, anemia of chronic disease, autoimmune hemolytic anemia, thrombocytosis, anemia due to GI bleed, and thrombosis.

Genitourinary manifestations include nephrolithiasis, obstructive uropathy, enterovesical fistulae, glomerulonephritis, and amyloidosis.

Pulmonary manifestations include granulomatous lung disease, fibrosing alveolitis, and pulmonary vasculitis.

Cardiovascular manifestations include pericarditis, myocarditis, and vasculitis

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Staging

Multiple scoring systems incorporating the patient’s history, physical findings, and laboratory data have been developed to assess disease activity in adults with Crohn disease. One such system is the Crohn Disease Activity Index (CDAI), which was developed for use in adults. The Pediatric Crohn Disease Activity Index (PCDAI) was developed and validated in 1990. Its results are correlated with the physician's global assessment and with the modified Harvey-Bradshaw index, and it has significant interobserver reliability. The important difference between this index and the CDAI is the inclusion of growth parameters in the score.

These scoring systems are used principally for assessing efficacy of treatment and evaluating new therapies for research purposes.

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Contributor Information and Disclosures
Author

Priya Rangasamy, MD  Fellow, Department of Gastroenterology/Hepatology, University of Connecticut Health Center

Priya Rangasamy, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Coauthor(s)

Yung-Hsin Chen, MD  Staff Physician, Department of Radiology, Nassau University Medical Center

Yung-Hsin Chen, MD is a member of the following medical societies: American Roentgen Ray Society, Radiological Society of North America, and Society of Skeletal Radiology

Disclosure: Nothing to disclose.

Marcy L Coash, DO  Staff Physician, Department of Internal Medicine, University of Connecticut

Marcy L Coash, DO is a member of the following medical societies: American Medical Student Association/Foundation and American Osteopathic Association

Disclosure: Nothing to disclose.

Spencer B Gay, MD  Professor of Radiology, Director of Body Computed Tomography, Department of Radiology, University of Virginia Health Sciences Center

Disclosure: Nothing to disclose.

John L Haddad, MD  Clinical Associate Professor, Department of Radiology, Weill Medical College of Cornell University; Director of Body MRI, Department of Radiology, Methodist Hospital in Houston

John L Haddad, MD is a member of the following medical societies: American College of Radiology, American Medical Association, and Radiological Society of North America

Disclosure: Nothing to disclose.

Eugene C Lin, MD  Consulting Radiologist, Virginia Mason Medical Center; Clinical Assistant Professor of Radiology, University of Washington School of Medicine

Eugene C Lin, MD is a member of the following medical societies: American College of Nuclear Medicine, American College of Radiology, Radiological Society of North America, and Society of Nuclear Medicine

Disclosure: Nothing to disclose.

Dermot PB McGovern, MD, PhD, MRCP  Director of Translational Medicine, Inflammatory Bowel and Immunobiology Research Institute; Associate Professor of Medicine, David Geffen School of Medicine, UCLA, Cedars-Sinai Medical Center

Dermot PB McGovern, MD, PhD, MRCP is a member of the following medical societies: American Gastroenterological Association, American Society of Human Genetics, British Society of Gastroenterology, and Royal College of Physicians of the United Kingdom

Disclosure: UCB Honoraria Consulting; UCB Honoraria Speaking and teaching; Salix Honoraria Speaking and teaching; UCB Grant/research funds Other; Prometheus Consulting fee Consulting

Gil Y Melmed, MD  Director, Clinical Trials, Inflammatory Bowel Disease Center, Cedars-Sinai Medical Center; Assistant Clinical Professor of Medicine, David Geffen School of Medicine at UCLA

Gil Y Melmed, MD is a member of the following medical societies: American College of Gastroenterology and American Gastroenterological Association

Disclosure: Amgen Consulting fee Consulting; Shire Honoraria Speaking and teaching; Proctor and Gamble Honoraria Speaking and teaching; UCB Consulting fee Consulting; Centocor Consulting fee Consulting; Prometheus Labs Honoraria Speaking and teaching

David I Weltman, MD  Consulting Staff, S & D Medical, LLP; Director, Department of Radiology, Southside Hospital

David I Weltman, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Association of Program Directors in Radiology, New York County Medical Society, and Radiological Society of North America

Disclosure: Nothing to disclose.

George Y Wu, MD, PhD  Professor, Department of Medicine, Director, Hepatology Section, Herman Lopata Chair in Hepatitis Research, University of Connecticut School of Medicine

George Y Wu, MD, PhD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, American Medical Association, American Society for Clinical Investigation, and Association of American Physicians

Disclosure: Springer Consulting fee Consulting; Gilead Consulting fee Review panel membership; Gilead Honoraria Speaking and teaching; Bristol-Myers Squibb Honoraria Speaking and teaching; Springer Royalty Review panel membership

Dahua Zhou, MD  Staff Physician, Department of Radiology, Nassau University Medical Center

Dahua Zhou, MD is a member of the following medical societies: Radiological Society of North America

Disclosure: Nothing to disclose.

Specialty Editor Board

Waqar A Qureshi, MD  Associate Professor of Medicine, Chief of Endoscopy, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine and Veterans Affairs Medical Center

Waqar A Qureshi, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

BS Anand, MD  Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine

BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Bruce Buehler, MD  Professor, Department of Pediatrics and Genetics, Director RSA, University of Nebraska Medical Center

Bruce Buehler, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Pediatrics, American Association on Mental Retardation, American College of Medical Genetics, American College of Physician Executives, American Medical Association, and Nebraska Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Kathleen M Raynor, MD, Priyankha Balasundaram, MD, and Senthil Nachimuthu, MD, FACP, to the development and writing of the source articles.

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Colonoscopic image of a large ulcer and inflammation of the descending colon in a 12-year-old boy with Crohn disease.
Histologic features of chronic colitis with crypt atrophy and branching, and lymphocytic infiltrate. Hematoxylin-eosin staining. Courtesy of Dr E. Ruchelli.
Colonic granuloma in a patient with Crohn disease. Hematoxylin-eosin staining. Courtesy of Dr E. Ruchelli.
Postoperative photograph depicts the incisions used for laparoscopic ileocolectomy in a 14-year-old male adolescent with obstruction of the terminal ileum. Note the 2-cm incision in the right lower abdomen through which the specimen was extracted and the extracorporeal anastomosis performed. The 12-mm umbilical incision is nicely hidden in the depths of the umbilicus. A 5-mm incision is visible in the left lower abdomen, and another is in the left suprapubic region just above the top of the pants.
On this laparoscopic photograph, the mesentery of the terminal ileum is being coagulated with a sealing device (LigaSure; Valley Lab, Boulder, Colo). Note that the ligation of the mesentery proceeds near the border of the ileum rather than at the base of the mesentery.
Image obtained during upper GI series with a small bowel follow-through shows narrowing and irregularity in the distal ileum in a 16-year-old male adolescent with Crohn disease.
Crohn disease. Aphthous ulcers. Double-contrast barium enema examination in Crohn colitis demonstrates numerous aphthous ulcers.
Crohn disease. Crohn colitis. Double-contrast barium enema study demonstrates marked ulceration, inflammatory changes, and narrowing of the right colon
Crohn disease. Cobblestoning. Spot view of the terminal ileum from a small bowel follow-through study demonstrates linear longitudinal and transverse ulcerations that create a cobblestone appearance. Also, note the relatively greater involvement of the mesenteric side of the terminal ileum and the displacement of the involved loop away from the normal small bowel secondary to mesenteric inflammation and fibrofatty proliferation.
Crohn disease of the terminal ileum. Small bowel follow-through study demonstrates the string sign in the terminal ileum. Also, note pseudodiverticula of the antimesenteric wall of the terminal ileum, secondary to greater distensibility of this less-involved segment of the wall.
Crohn disease. Spot view of the terminal ileum from a small bowel follow-through study demonstrates the string sign, consistent with narrowing and stricturing. Also, note a sinus tract originating from the medial wall of the terminal ileum and the involvement of the medial wall of the cecum.
Crohn disease. Enterocolic fistula. Double-contrast barium enema study demonstrates multiple fistulous tracts between the terminal ileum and the right colon adjacent to the ileocecal valve, the so-called double-tracking of the ileocecal valve.
Crohn disease. Active small bowel inflammation. CT scan demonstrates small bowel wall thickening, mesenteric inflammatory stranding, and mesenteric adenopathy.
MRI of an inflamed terminal ileum in a 10-year-old girl with Crohn disease.
MRI of a small abscess on the right side of the anal sphincter in a 9-year-old boy with Crohn disease.
Table. Characteristics Differentiating Crohn Disease and Ulcerative Colitis
Characteristic
Crohn diseaseUlcerative colitis
DistributionEntire GI tractColon only, although gastritis recognized
Skip lesionsContinuous involvement proximally from rectum
PathologyFull thicknessMucosa only
Granulomas (30%)No granulomas
RadiologyEntire GI tractColon only
Skip lesionsContinuous involvement proximally from rectum
Fistulae, abscesses, fibrotic stricturesMucosal disease only
Cancer riskIncreasedEstimated 1% per year starting 10 years after diagnosis
Presentation
Crohn diseaseUlcerative colitis
BleedingOccasionalVery common
ObstructionCommonUncommon
FistulaCommonNone
Weight lossCommonUncommon
Perianal diseaseCommonRare
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