Management of mild or moderate ulcerative colitis extending beyond the rectum in children

UC: ulcerative colitis; IV: intravenous; 5-ASA: 5-aminosalicylate; 6-MP: 6-mercaptopurine; AZA: azathioprine; PUCAI: pediatric ulcerative colitis activity index.
* Severity of disease is typically assessed by the PUCAI, which assigns a score based on symptoms of abdominal pain, rectal bleeding, stool consistency, stool frequency, presence of nocturnal stools, and activity level, adding to a maximum score of 85. Mild disease is defined by a PUCAI score of 10 to 34, moderate disease 35 to 64, and severe disease 65 to 85.
¶ The dotted line indicates that 5-ASA is a secondary option for inducing remission in patients with moderate UC. Most patients with moderate symptoms will benefit from a course of systemic glucocorticoids, especially if systemic symptoms such as fever or anorexia are present. However, for patients who are reluctant to use glucocorticoids, it is reasonable to offer a trial of 5-ASA, using doses at the high end of the range. Patients who do not improve on 5-ASA should move on to glucocorticoids.
Δ Another option used for selected patients is early use of a biologic agent (typically for hospitalized patients, using infliximab or adalimumab), similar to the approach for patients with more severe disease.
◊ Oral glucocorticoids are typically given for 2 to 4 weeks, then gradually tapered over the next 2 months. Either prednisone or budesonide may be used.
§ Response to therapy is typically determined by changes in clinical symptoms, documented by the PUCAI score. The goal of therapy is complete resolution of symptoms, reflected by a PUCAI score of <10. For patients with moderate or severe UC, a marked improvement in PUCAI (eg, PUCAI <35 or a PUCAI decrease of ≥20 points) provides an initial indication of successful medical therapy. Results of laboratory tests including fecal calprotectin should also be incorporated into a global assessment when making treatment decisions, interpreted in the context of the patient's historical results. In addition, colonoscopy is recommended before making major changes in therapy or when it is unclear whether symptoms are disease-related, especially if fecal calprotectin is elevated.
¥ IV glucocorticoids are typically given for up to 14 days, transitioning to oral steroids approximately 2 days after a definite response is noted. If there is no clear response after 1 week of IV therapy, the patient should be reevaluated by endoscopy and discussion initiated regarding other immunosuppressive therapy (thiopurines or infliximab). Some experts now routinely use endoscopic remission as a treatment goal and to determine when to transition from IV to oral therapy.
‡ Patients who relapse during treatment with 5-ASA may be reinduced with glucocorticoids. Patients are considered to be steroid-dependent if they have been on high-dose glucocorticoids for more than 2 to 3 months or daily glucocorticoids for 4 to 6 months, or frequently flare when the glucocorticoid dose is reduced (eg, 2 or 3 relapses per year). These patients should advance to a different immunosuppressive therapy.
† For initial medical treatment of patients with steroid-dependent/refractory disease, either a thiopurine, infliximab or adalimumab is a reasonable choice and the optimal approach has not been established. If thiopurines are selected, patients may require short-term "bridging" with another medication (corticosteroids, tacrolimus, or cyclosporine) due to the slow onset of action of thiopurines. For those who fail initial treatment with thiopurines, it is common practice to transition to infliximab or adalimumab therapy. Although this type of sequential therapy is common, the risks and benefits have not been assessed compared with proceeding to colectomy. For patients who fail initial treatment with infliximab or adalimumab, it is common practice to move on to second-line options rather than to a trial of thiopurines.
** For patients who relapse after initial success with a first- or second-line treatment for steroid-dependent or steroid-refractory colitis, colectomy should be seriously considered. The alternatives are either sequential treatment with other biologic agents, or treatment with other immunosuppressive agents in which there is only limited evidence of benefit (thalidomide) or experimental agents (eg, tofacitinib).