Introduction

Crohn's disease is a form of inflammatory bowel disease. It commonly affects the terminal ileum and colon but may be seen anywhere from the mouth to anus.

Epidemiology

  • Incidence: 11.00 cases per 100,000 person-years
  • Peak incidence: 20-30 years
  • Sex ratio: 1:1
Condition Relative
incidence
Coeliac disease9.09
Colorectal cancer5.82
Crohn's disease1
Microscopic colitis0.91
Ulcerative colitis0.91
<1 1-5 6+ 16+ 30+ 40+ 50+ 60+ 70+ 80+

Pathophysiology

Pathology
  • cause is unknown but there is a strong genetic susceptibility
  • inflammation occurs in all layers, down to the serosa. This is why patients with Crohn's are prone to strictures, fistulas and adhesions

Clinical features

Crohn's disease typically presents in late adolescence or early adulthood. Features include:
  • presentation may be non-specific symptoms such as weight loss and lethargy
  • diarrhoea: the most prominent symptom in adults. Crohn's colitis may cause bloody diarrhoea
  • abdominal pain: the most prominent symptom in children
  • perianal disease: e.g. Skin tags or ulcers
  • extra-intestinal features are more common in patients with colitis or perianal disease

Investigations

Crohn's disease is a form of inflammatory bowel disease. It commonly affects the terminal ileum and colon but may be seen anywhere from the mouth to anus

Bloods
  • C-reactive protein correlates well with disease activity

Endoscopy
  • colonoscopy is the investigation of choice
  • features suggest of Crohn's include deep ulcers, skip lesions

Histology
  • inflammation in all layers from mucosa to serosa
  • goblet cells
  • granulomas

Small bowel enema
  • high sensitivity and specificity for examination of the terminal ileum
  • strictures: 'Kantor's string sign'
  • proximal bowel dilation
  • 'rose thorn' ulcers
  • fistulae

© Image used on license from Radiopaedia
Barium study is shown from a patient with worsening Crohn's disease. Long segment of narrowed terminal ileum in a 'string like' configuration in keeping with a long stricture segment. Termed 'Kantor's string sign'.

Differential diagnosis

The two main types of inflammatory bowel disease are Crohn's disease and ulcerative colitis. They have many similarities in terms of presenting symptoms, investigation findings and management options.

Venn diagram showing shared features and differences between ulcerative colitis and Crohn's disease. Note that whilst some features are present in both, some are much more common in one of the conditions, for example colorectal cancer in ulcerative colitis

There are however some key differences which are highlighted in table below:

Crohn's disease (CD)Ulcerative colitis (UC)
FeaturesDiarrhoea usually non-bloody
Weight loss more prominent
Upper gastrointestinal symptoms, mouth ulcers, perianal disease
Abdominal mass palpable in the right iliac fossa
Bloody diarrhoea more common
Abdominal pain in the left lower quadrant
Tenesmus
Extra-intestinalGallstones are more common secondary to reduced bile acid reabsorption

Oxalate renal stones*
Primary sclerosing cholangitis more common
ComplicationsObstruction, fistula, colorectal cancerRisk of colorectal cancer high in UC than CD
PathologyLesions may be seen anywhere from the mouth to anus

Skip lesions may be present
Inflammation always starts at rectum and never spreads beyond ileocaecal valve

Continuous disease
HistologyInflammation in all layers from mucosa to serosa
  • increased goblet cells
  • granulomas
No inflammation beyond submucosa (unless fulminant disease) - inflammatory cell infiltrate in lamina propria
  • neutrophils migrate through the walls of glands to form crypt abscesses
  • depletion of goblet cells and mucin from gland epithelium
  • granulomas are infrequent
EndoscopyDeep ulcers, skip lesions - 'cobble-stone' appearanceWidespread ulceration with preservation of adjacent mucosa which has the appearance of polyps ('pseudopolyps')
RadiologySmall bowel enema
  • high sensitivity and specificity for examination of the terminal ileum
  • strictures: 'Kantor's string sign'
  • proximal bowel dilation
  • 'rose thorn' ulcers
  • fistulae
Barium enema
  • loss of haustrations
  • superficial ulceration, 'pseudopolyps'
  • long standing disease: colon is narrow and short -'drainpipe colon'

*impaired bile acid rebsorption increases the loss calcium in the bile. Calcium normally binds oxalate.

Management

Crohn's disease is a form of inflammatory bowel disease. It commonly affects the terminal ileum and colon but may be seen anywhere from the mouth to anus. NICE published guidelines on the management of Crohn's disease in 2012.

General points
  • patients should be strongly advised to stop smoking
  • some studies suggest an increased risk of relapse secondary to NSAIDs and the combined oral contraceptive pill but the evidence is patchy

Inducing remission
  • glucocorticoids (oral, topical or intravenous) are generally used to induce remission. Budesonide is an alternative in a subgroup of patients
  • enteral feeding with an elemental diet may be used in addition to or instead of other measures to induce remission, particularly if there is concern regarding the side-effects of steroids (for example in young children)
  • 5-ASA drugs (e.g. mesalazine) are used second-line to glucocorticoids but are not as effective
  • azathioprine or mercaptopurine* may be used as an add-on medication to induce remission but is not used as monotherapy. Methotrexate is an alternative to azathioprine
  • infliximab is useful in refractory disease and fistulating Crohn's. Patients typically continue on azathioprine or methotrexate
  • metronidazole is often used for isolated peri-anal disease

Maintaining remission
  • as above, stopping smoking is a priority (remember: smoking makes Crohn's worse, but may help ulcerative colitis)
  • azathioprine or mercaptopurine is used first-line to maintain remission
  • methotrexate is used second-line
  • 5-ASA drugs (e.g. mesalazine) should be considered if a patient has had previous surgery

Surgery
  • around 80% of patients with Crohn's disease will eventually have surgery
  • see below for further detail


Surgical interventions in Crohn's disease

The commonest disease pattern in Crohn's is stricturing terminal ileal disease and this often culminates in an ileocaecal resection. Other procedures performed include segmental small bowel resections and stricturoplasty. Colonic involvement in patients with Crohn's is not common and, where found, distribution is often segmental. However, despite this distribution segmental resections of the colon in patients with Crohn's disease are generally not advocated because the recurrence rate in the remaining colon is extremely high, as a result, the standard options of colonic surgery in Crohn's patients are generally; sub total colectomy, panproctocolectomy and staged sub total colectomy and proctectomy. Restorative procedures such as ileoanal pouch have no role in therapy.

Crohn's disease is notorious for the developmental of intestinal fistulae; these may form between the rectum and skin (perianal) or the small bowel and skin. Fistulation between loops of bowel may also occur and result in bacterial overgrowth and malabsorption. Management of enterocutaneous fistulae involves controlling sepsis, optimising nutrition, imaging the disease and planning definitive surgical management.

*assess thiopurine methyltransferase (TPMT) activity before offering azathioprine or mercaptopurine

Complications

As well as the well-documented complications described above, patients are also at risk of: