3 Extraintestinal manifestations and complications

The many systemic associations and complications of IBD affect 6–50% of patients with the disease; most affect the liver/biliary tree, joints, skin and eyes (Table 3.1). Most occur in patients with colitis and some largely in those with active disease. In some instances (ankylosing spondylitis [AS], uveitis, arthropathy), there seems to be a genetic and/or immunologic association with IBD: these associations are often termed extraintestinal manifestations. Others are complications of IBD; for example, anemia and metabolic problems (gallstones and urinary stones).

Hepatobiliary manifestations

Sclerosing cholangitis occurs in about 5% of patients with ulcerative colitis and a smaller proportion of those with Crohn’s disease. The pathogenesis is unknown, but the condition may occur years before the onset of overt colitis; 80% of patients have perinuclear antineutrophil cytoplasmic antibodies (pANCAs) in their serum. The condition is characterized by the gradual progression of an inflammatory obliterative fibrosis of the extra- and intrahepatic biliary tree (Figure 3.1), and is sometimes complicated by cholangiocarcinoma. The risk of colorectal cancer in patients with ulcerative colitis and sclerosing cholangitis exceeds that associated with ulcerative colitis alone.

Patients usually present with complications of biliary stricturing, such as obstructive jaundice, cholangitis or abnormal liver-function tests (raised alkaline phosphatase and γ-glutamyltranspeptidase) at routine screening. The diagnosis may be suggested by ultrasound and is usually confirmed by magnetic resonance cholangiopancreatography (MRCP) and/or liver biopsy; use of endoscopic retrograde cholangiopancreatography (ERCP) (see Figure 3.1) is now largely confined to the stenting of dominant strictures.

The course of sclerosing cholangitis is steadily progressive. Oral ursodeoxycholic acid in standard doses (10–15 mg/kg/day) improves pruritus and jaundice, but, paradoxically, high doses (25–30 mg/kg/day) may worsen outcome; its previously suspected beneficial effect on the incidence of colorectal cancer in patients with ulcerative colitis and sclerosing cholangitis has recently been called into question. Liver transplant is the only hope of long-term survival in those who do not develop cholangiocarcinoma; otherwise, median survival for symptomatic patients is about 15 years.

Patients with sclerosing cholangitis should be screened annually by colonoscopy and MRCP for colorectal and bile duct cancer, respectively.

Pauciarticular disease involves fewer than five joints; characteristically, it affects one large joint, for example the knee, and is most common in women. Attacks usually coincide with relapse of colitis; sometimes there is simultaneous erythema nodosum or iritis (see below). Its pathogenesis may involve deposition of gut-derived immune complexes in the affected joint in genetically predisposed individuals. Although the attacks of arthritis may come and go over many years, the disease is neither progressive nor deforming. In most people, the joint symptoms resolve on treatment of the active colitis. Sulfasalazine may be more effective for the joints than other aminosalicylates. Acetylsalicylic acid (ASA; aspirin) and other non-steroidal anti-inflammatory drugs (NSAIDs) may exacerbate IBD (see Table 1.1, page 9) and therefore should be avoided if possible. Alternatives include paracetamol (acetaminophen) and joint aspiration with steroid instillation.

Polyarticular IBD-related arthropathy affects more than five joints, particularly small joints such as the metacarpophalangeals, and can often be symmetrical. Symptoms are more common in women, are chronic and are not clearly related to activity of the associated IBD. It may sometimes be misdiagnosed as rheumatoid arthritis. Management resembles that of pauciarticular disease, except that response of the arthropathy to treatment of the IBD itself is poor.

Ankylosing spondylitis and sacroiliitis. While about 95% of patients without IBD who have AS are HLA-B27 positive, this is true of only 50–80% of those with both diseases. AS affects about 5% of patients with ulcerative or Crohn’s colitis and, like enteropathic arthritis, it is probably immunologically mediated. The patient presents with back pain, stiffness and, in the later stages of the disease, kyphosis (Figure 3.2); diagnosis is confirmed by radiography. The course of AS is independent of the activity of IBD, and it may present years before the bowel disease manifests. Treatment consists of vigorous physiotherapy, sulfasalazine and, if tolerated, NSAIDs. Anti-tumor necrosis factor (TNF)α agents (see Chapter 5) are effective in refractory AS.

Sacroiliitis can be an early symptom of AS or can occur in isolation. It presents with low backache and morning stiffness, although it can also be asymptomatic. In contrast to mechanical back pain, it is insidious in onset, occurring in younger people. MRI is more sensitive than plain X-ray for diagnosis. Treatment follows the same principles as for AS.

Related posts:

Principles of management Prognosis Surgery Medical management of ulcerative colitis Medical management of Crohn’s disease Diagnosis

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