granulomas; affected areas may occur in any part of the
gastro-intestinal tract, interspersed with areas of relatively
normal tissue. Crohn’s disease may present as recurrent
attacks, with acute exacerbations combined with periods of
remission or less active disease. Symptoms depend on the
site of disease but may include abdominal pain, diarrhoea,
fever, weight loss, and rectal bleeding.
Complications of Crohn’s disease include intestinal
strictures, abscesses in the wall of the intestine or adjacent
structures,fistulae, anaemia, malnutrition, colorectal and
small bowel cancers, and growth failure and delayed puberty
in children. Crohn’s disease may also be associated with
extra-intestinal manifestation: the most common are
arthritis and abnormalities of the joints, eyes, liver and skin.
Crohn’s disease is also a cause of secondary osteoporosis and
those at greatest risk should be monitored for osteopenia
and assessed for the risk of fractures.
Up to a third of patients with Crohn’s disease are
diagnosed before the age of 21 years but there is a lack of
evidence regarding treatment for children. Paediatric
practice is often based on extrapolation from adult studies.Fistulating Crohn’s disease
Fistulating Crohn’s disease is a complication that involves
the formation of afistula between the intestine and adjacent
structures, such as perianal skin, bladder, and vagina. It
occurs in about one quarter of patients, mostly when the
disease involves the ileocolonic area.Aims of treatment
Treatment is largely directed at the induction and
maintenance of remission and the relief of symptoms. Active
treatment of acute Crohn’s disease should be distinguished
from preventing relapse. The aims of drug treatment are to
reduce symptoms and maintain or improve quality of life,
while minimising toxicity related to drugs over both the
short and long term. Drug treatment should always be
initiated by a paediatric gastroenterologist.
Infistulating Crohn’s disease, surgery and medical
treatment aim to close and maintain closure of thefistula.Non-drug treatment
gIn addition to drug treatment, management options for
Crohn’s disease include smoking cessation and attention to
nutrition, which plays an important role in supportive care.
Surgery may be considered in certain children with early
disease limited to the distal ileum and in severe or chronic
active disease.hDrug treatment
Treatment of acute disease
Monotherapy
gA corticosteroid (either prednisolone p. 442 or
methylprednisolone p. 441 or intravenous hydrocortisone
p. 440 ), is used to induce remission in children with afirst
presentation or a single inflammatory exacerbation of
Crohn’s disease in a 12 -month period.
Enteral nutrition is an alternative to a corticosteroid when
there is concern about growth or side effects.
In children with distal ileal, ileocaecal or right-sided
colonic disease, in whom a conventional corticosteroid is
unsuitable or contra-indicated, budesonide p. 32
[unlicensed] may be considered. Budesonide is less effective
but may cause fewer side-effects than other corticosteroids,
as systemic exposure is limited. Aminosalicylates (such as
sulfasalazine p. 31 and mesalazine p. 29 ) are an alternative
option in these children. They are less effective than a
corticosteroid or budesonide [unlicensed], but may be
preferred because they have fewer side-effects.
Aminosalicylates and budesonide are not appropriate for
severe presentations or exacerbations.hAdd-on treatment
gAdd on treatment is prescribed if there are two or more
inflammatory exacerbations in a 12 -month period, or the
corticosteroid dose cannot be reduced.
Azathioprine p. 518 or mercaptopurine p. 543 [unlicensed
indications] can be added to a corticosteroid or budesonide
to induce remission. In children who cannot tolerate
azathioprine or mercaptopurine or in whom thiopurine
methyltransferase (TPMT) activity is deficient, methotrexate
p. 543 can be added to a corticosteroid.
Under specialist supervision, monoclonal antibody
therapies, adalimumab p. 642 and infliximab p. 33 ,are
options for the treatment of severe, active Crohn’s disease,
following inadequate response to conventional therapies or
in those who are intolerant of or have contra-indications to
conventional therapy.hSee alsoNational funding/access
decisionsfor adalimumab and infliximab.
gAdalimumab and infliximab can be used as
monotherapy or combined with an immunosuppressant,
although there is uncertainty about the comparative
effectiveness.hThere are concerns about the long-term
safety of adalimumab and infliximab in children;
malignancies, including hepatosplenic T- cell lymphoma,
have been reported.
Maintenance of remission
gChildren, and their parents or carers, should be made
aware of the risk of relapse with and without drug treatment,
and symptoms that may suggest a relapse (most frequently
unintended weight loss, abdominal pain, diarrhoea and
general ill-health). For those who choose not to receive
maintenance treatment during remission, a suitable follow
up plan should be agreed upon and information provided on
how to access healthcare if a relapse should occur.
Azathioprine or mercaptopurine [unlicensed indications]
as monotherapy can be used to maintain remission when
previously used with a corticosteroid to induce remission.
They may also be used in children who have not previously
received these drugs (particularly those with adverse
prognostic factors such as early age of onset, perianal
disease, corticosteroid use at presentation, and severe
presentations). Methotrexate [unlicensed] can be used to
maintain remission only in children who required
methotrexate to induce remission, or who are intolerant of
or are not suitable for azathioprine or mercaptopurine for
maintenance. Corticosteroids or budesonide should not be
used.hMaintaining remission following surgery
gAzathioprine or mercaptopurine can be considered to
maintain remission after surgery in children with adverse
prognostic factors such as more than one resection, or
previously complicated or debilitating disease (for example,
abscess, involvement of adjacent structures,fistulating or
penetrating disease). Aminosalicylates can also be
considered as an option, however budesonide or enteral
nutrition should not be used.h
Other treatments
gLoperamide hydrochloride p. 49 can be used to manage
diarrhoea associated with Crohn’s disease in children who do
not have colitis.hColestyramine p. 129 is licensed for the
relief of diarrhoea associated with Crohn’s disease. See also
Diarrhoea (acute) p. 47.Fistulating Crohn’s disease
Perianalfistulae are the most common occurrence in
children withfistulating Crohn’s disease.gTreatment
may not be necessary for simple, asymptomatic perianal
fistulae. Whenfistulae are symptomatic, local drainage and
surgery may be required in conjunction with medical
therapy.
Metronidazole p. 333 or ciprofloxacin p. 348 [unlicensed
indications], alone or in combination, can improve
symptoms offistulating Crohn’s disease but complete26 Chronic bowel disorders BNFC 2018 – 2019
Gastro-intestinal system1