What are the extraintestinal manifestations in Inflammatory Bowel Disease (IBD)?

From the Guidelines

Extraintestinal manifestations (EIMs) in inflammatory bowel disease (IBD) should be managed with a multidisciplinary approach, considering the specific manifestation and its relationship with intestinal disease activity, as outlined in the most recent guidelines 1.

Overview of EIMs in IBD

EIMs are common complications of IBD, affecting up to 40% of patients with Crohn's disease or ulcerative colitis. These manifestations can involve multiple organ systems beyond the gastrointestinal tract, including musculoskeletal, dermatological, ocular, and hepatobiliary systems.

Common EIMs in IBD

The most common EIMs include:

• Musculoskeletal conditions such as peripheral arthritis, axial arthropathy, and ankylosing spondylitis
• Dermatological manifestations like erythema nodosum and pyoderma gangrenosum
• Ocular complications including episcleritis and uveitis
• Hepatobiliary disorders, particularly primary sclerosing cholangitis

Management of EIMs

Treatment approaches depend on the specific manifestation but often involve managing the underlying IBD with medications such as:

• Corticosteroids (e.g., prednisone 40-60mg daily with taper)
• Immunomodulators (e.g., azathioprine 2-3mg/kg/day or methotrexate 15-25mg weekly)
• Biologics (e.g., infliximab 5mg/kg at weeks 0, 2, 6, then every 8 weeks; adalimumab 160mg initially, 80mg at week 2, then 40mg every other week)

Specific Considerations

Some EIMs, like peripheral arthritis, typically parallel intestinal disease activity and improve with IBD treatment, while others, like ankylosing spondylitis and primary sclerosing cholangitis, follow independent courses requiring specific management. Early recognition of EIMs is crucial as they can significantly impact quality of life and sometimes precede intestinal symptoms, potentially aiding in earlier IBD diagnosis. According to recent guidelines, the management of complex EIMs should be discussed in a multidisciplinary team meeting 2. Additionally, anaemia is a common EIM in IBD, and its management is crucial to improve quality of life and reduce morbidity and mortality 3.

From the Research

Prevalence of Extraintestinal Manifestations in IBD

• Extraintestinal manifestations (EIMs) can occur in up to 24% of patients with IBD before the onset of intestinal symptoms 4
• EIMs can occur in up to one third of patients with Crohn's disease or ulcerative colitis 5
• As many as 36% of patients with IBD have at least one EIM 6

Organs Affected by Extraintestinal Manifestations

• EIMs most frequently affect joints, skin, or eyes, but can also affect other organs, such as the liver, lung, and pancreas 4
• Dermatologic and musculoskeletal manifestations are the most common EIMs 5
• Nearly every organ can be involved in EIMs, including the liver, lung, pancreas, and others 5, 6

Relationship between Intestinal Inflammation and Extraintestinal Manifestations

• Peripheral arthritis, oral aphthous ulcers, episcleritis, or erythema nodosum can be associated with active intestinal inflammation and can improve on standard treatment of the intestinal inflammation 4
• Anterior uveitis, ankylosing spondylitis, and primary sclerosing cholangitis usually occur independent of disease flares 4
• EIMs may either parallel intestinal inflammation or be completely independent of disease activity 5

Diagnosis and Treatment of Extraintestinal Manifestations

• A specialist's expertise is needed to confirm the diagnosis of extraintestinal manifestations before the inclusion of a patient in IBD trials, except for axial spondyloarthritis, for which typical symptoms and MRI can be sufficient 7
• Easy-to-measure endpoints were identified to assess the response of extraintestinal manifestations to treatment without needing specialist involvement 7
• The pharmacological management of IBD has been expanded and refined to include 5 main drug classes, including 5-aminosalicylates, corticosteroids, immunomodulators, biologics, and antibiotics 8

Assessment of Extraintestinal Manifestations in IBD Trials

• The EXTRA consensus proposes guidelines on how to thoroughly evaluate extraintestinal manifestations within IBD trials 7
• The guidelines recommend that specialist expertise is needed to confirm the diagnosis of extraintestinal manifestations and to assess the response to treatment for certain EIMs, such as uveitis, peripheral spondyloarthritis, and arthralgia 7