Inflammatory bowel disease: problems outside the intestines

Extraintestinal manifestations of IBD may indicate diagnosis or treatment adjustment.

Inflammatory bowel disease (IBD) is a form of chronic inflammation of the gastrointestinal tract. Ulcerative colitis, which is characterized by continuous inflammation of the colon, and Crohn's disease, which causes patches of inflammation and can occur anywhere in the gastrointestinal tract, are both types of IBD. Patients with IBD may experience a variety of gastrointestinal symptoms such as abdominal pain, diarrhea, blood in stool, bloating, and weight loss.

What non-gastrointestinal diseases can occur in people with IBD?

IBD symptoms can occur both inside and outside the gastrointestinal tract. The latter are known as extraintestinal manifestations (EIMs) of IBD. These conditions occur in almost half of all people with IBD and can occur before and/or after the diagnosis of IBD. EIMs are more common in people who receive their diagnosis at an earlier age and also tend to occur earlier in the course of IBD. In fact, approximately 24% of EIM symptoms are present before an IBD diagnosis is made. The causes of EIM are poorly understood but, similar to IBD, may be due to a combination of genetic risk factors, immune responses, and lifestyle factors such as cigarette smoking.

What are some examples of extraintestinal manifestations of IBD?

Patients can have multiple EIMs of varying severity at the same time. EIMs may not be easy to recognize, and almost any organ system can be affected. Sometimes the severity of EIM symptoms mirrors that of intestinal symptoms, but under certain conditions the EIMs behave independently. Below are some common examples of extraintestinal manifestations.

Musculoskeletal system:Musculoskeletal manifestations of IBD are the most common, occurring in up to 46% of IBD patients. They can manifest as inflammatory back pain (ankylosing spondylitis), tendon or ligament inflammation, arthritis, joint pain without arthritis, or swelling of the fingers or toes (dactylitis). Arthritis can occur both within the axial skeleton (hips, lower back, spine) and peripherally (fingers, wrists, elbows, knees, ankles).

Mucous membrane:IBD can cause changes in the skin and mucous membranes (the moist lining that covers certain organs and body cavities). Oral ulcers (seen in Crohn's disease), erythema nodosum (raised purple nodules typically on the front of the legs that can occur in 10% to 15% of patients), and pyoderma gangrenosum (painful skin ulcers) are some examples of how IBD can affect the skin. Sweet's syndrome, which involves tender nodules under the skin and is also associated with increased white blood cell counts, fever, arthritis and eye symptoms, is a rare EIM.

Eyepiece:Inflammation in parts of the eye (episcleritis, scleritis, or anterior uveitis) affects 2% to 7% of IBD patients. If patients experience eye pain, redness, tenderness, or visual disturbances, an urgent evaluation by an ophthalmologist may be required due to the risk of blindness from uncontrolled inflammation.

Vessel:Patients with IBD are up to three times more likely to develop blood clots compared to patients without IBD. Sometimes these clots can travel to the lungs and are called a pulmonary embolism. Symptoms of blood clots may include leg swelling or shortness of breath. Poorly controlled inflammation in the gastrointestinal tract is thought to be the cause of an increased risk of clotting.

Gastrointestinal:Although not common, patients can develop liver diseases related to IBD, including primary sclerotic cholangitis (inflammation and scarring of the bile ducts) and autoimmune hepatitis (when the immune system attacks the liver cells, causing liver inflammation). Autoimmune pancreatitis has also been reported. These conditions can be diagnosed based on symptoms, blood tests, or imaging findings (sometimes with MRI).

How are EIMs treated?

It is important to effectively treat intestinal inflammation as this can reduce the activity of extraintestinal manifestations. Sometimes EIMs may require additional specific treatments. For example, corticosteroids, sulfasalazine (anti-inflammatory drugs), or specific biologic therapies may be considered for the treatment of arthritis. Skin and ocular manifestations of IBD can be treated with topical or systemic corticosteroids, immunosuppressants, or specific biologic therapies. Vascular manifestations such as blood clots can be treated with anticoagulant medications. The treatment of EIMs is complex and often requires a collaborative treatment approach with multiple healthcare providers.

What should I do if I have symptoms?

It's important to recognize EIMs because they can significantly impact your quality of life and, over time, can also impact your doctor's approach to IBD treatment and monitoring.

Whether you have been diagnosed with IBD or not, it is important to see your primary care doctor or gastroenterologist (if you have one) to discuss any symptoms you may be experiencing. Your doctor(s) will discuss your medical and family history to determine your risk for IBD or other conditions.

For patients with a diagnosis of IBD or at risk of IBD, EIMs may be the first indication of the diagnosis or the need for treatment review and adjustment. It is important to communicate both your gastrointestinal and non-gastrointestinal symptoms to your gastroenterologist so that appropriate treatment can begin promptly and connected with specialists in the affected organ system. Lifestyle changes such as quitting smoking can also reduce the risk of EIM.

With targeted treatment and the appropriate on-site healthcare team, management of both IBD and EIM can be achieved to improve patients' quality of life.