Extraintestinal Manifestations of Inflammatory Bowel Disease

Abstract

Extraintestinal manifestations (EIM) in inflammatory bowel disease (IBD) are frequent and may occur before or after IBD diagnosis. EIM may impact the quality of life for patients with IBD significantly requiring specific treatment depending on the affected organ(s). They most frequently affect joints, skin, or eyes, but can also less frequently involve other organs such as liver, lungs, or pancreas. Certain EIM, such as peripheral arthritis, oral aphthous ulcers, episcleritis, or erythema nodosum, are frequently associated with active intestinal inflammation and usually improve by treatment of the intestinal activity. Other EIM, such as uveitis or ankylosing spondylitis, usually occur independent of intestinal inflammatory activity. For other not so rare EIM, such as pyoderma gangrenosum and primary sclerosing cholangitis, the association with the activity of the underlying IBD is unclear. Successful therapy of EIM is essential for improving quality of life of patients with IBD. Besides other options, tumor necrosis factor antibody therapy is an important therapy for EIM in patients with IBD.

FIGURE 1

A, Oral aphthous ulcers, (B) Sweet's syndrome, (C) erythema nodosum, (D) pyoderma gangrenosum, (E) peristomal pyoderma gangrenosum, (F) episcleritis, (G) uveitis with hypopyon and dilated iris vessels, (H) conventional x-ray of the lateral spine demonstrating syndesmophytes (bamboo spine), (I) plane radiograph of the ileosacral joints with bilateral sacroiliitis, (J) plane radiography of the sacrum with bilateral ankylosis, (K) coronal magnetic resonance image of the sacroiliac joints with active inflammation mainly on the left side and chronic inflammatory changes on both sides.

FIGURE 2

Chronology of EIM in patients with IBD. In one quarter of patients with IBD, up to 4 EIM appeared before the time of IBD diagnosis. The median time before IBD diagnosis is 5 mo (range, 0–25 mo). In 75% of cases, the first EIM manifested after IBD diagnosis (median, 92 mo; range, 29–183 mo). Thirty years after diagnosis up to 50% of patients with IBD have suffered from at least 1 EIM.

FIGURE 3

Unpublished data from the Swiss IBD cohort study. Location of erythema nodosum in male and female patients suffering from IBD.

FIGURE 4

Unpublished data from the Swiss IBD cohort study. Location of pyoderma gangrenosum in male and female patients suffering from IBD.