Crohn's disease in children & adolescents

Crohn's disease in children and adolescents

Refer to adult algorithms for detailed care pathways, whose principles usually apply to pediatrics

ECCO Statement 10A (2010)

Children and adolescents with suspected IBD require a thorough history and examination, including assessment of growth velocity and pubertal stage [EL4, RGC]. Normal laboratory investigations do not exclude a diagnosis of IBD [EL 2b, RG B]. Normal levels of faecal surrogate markers for intestinal inflammation, such as calprotectin or lactoferrin, make active disease in the lower gastrointestinal tract unlikely and may guide the need for invasive investigation [EL 3b, RG B].

Clinical assessment

Consider need to exclude primary immunodeficiency

Antibiotics and per-cutaneous (possibly surgical) drainage

ECCO Statement 10B (2010)

Initial investigation should consist of colonoscopy (including terminal ileal intubation) with multiple biopsies [EL2b, RG B], upper GI endoscopy with multiple biopsies [EL2b, RGB], and small bowel imaging [EL2b, RGB]. The technique used to examine the small bowel will depend on local expertise;but dynamic contrast-enhanced magnetic resonance imaging can reliably show most lesions of Crohn's disease without exposure to ionizing radiation [EL 2b, RG C].

Further information

  • Arrange blood testing, faecal biomarkers and microbiology (CDT, MC&S, parasites if travel history); ileo-colonoscopy and gastroscopy with biopsies during general anaesthesia; and oral contrast small bowel MRI. Evaluate need for colonoscopy if faecal biomarkers normal
  • Patency then capsule endoscopy indicated if suspicion of disease remains high; likely safe beyond infancy
Investigations

ECCO Statement 10M (2010)

Nutritional status, growth and pubertal development should be recorded at diagnosis and during the course of disease. Nutritional deficiencies should be vigorously treated [EL3, RG B].

Further information

  • Bone density results difficult to interpret in the growth delayed child and need to be repeated to be meaningful
  • If bone density low, optimize weight bearing exercise, nutrition including vitamin D and Ca++, and Crohn's disease treatment
  • 10-40% have growth/pubertal delay: assess growth velocity at every visit. Intensify treatment and optimise calories if growth delay
  • Provide nutritional support if required
Measure bone density and growth velocity

ECCO Statement 10N (2010)

The care of children with CD should involve a multi-disciplinary team in a paediatric gastroenterology centre [EL5, RGD]. Transition clinics for adolescents with Crohn's disease represent optimal care and are highly recommended [EL5, RG D].

Refer to paediatric GI centre

Crohn's disease confirmed

ECCO Statement 10L (2010)

Psychosocial support should be given to patients and their families [EL4, RG C].

Provide ongoing psychosocial support

  • Mild disease represents CDAI 150-220, equivalent to Harvey-Bradshaw score of 5-6
Quiescent or mild disease

Moderate or severe disease

Localized (stricturing) or treatment-resistant disease

ECCO Statement 10K (2010)

Elective surgery should be considered in children with disease resistant to medical therapies, especially in pre-pubertal or early pubertal children with growth failure and localized Crohn's disease [EL4 RGC].

ECCO statement 6B (CD 2016)

If a patient has a relapse, escalation of the maintenance treatment can be considered to prevent disease progression [EL2]. Steroids should not be used to maintain remission [EL1]. Surgery should always be considered as an option in localized disease [EL4]

Further information


Surgery

Quiescent or mild disease

Quiescent or mild disease

ECCO Statement 10E (2010)

The role of mesalazine [EL2b, RG B], antibiotics [EL4 RGD] and probiotics [EL4, RGC] for inducing remission in children with active CD is unclear.

ECCO Statement 10G (2010)

The role of mesalazine in maintaining remission in paediatric Crohn's disease is unclear [EL2b, RG B].

Further information

  • There is no paediatric trial data supporting mesalazine as induction therapy; slight benefit in adult studies. Few side effects. Possible role in preventing colonic neoplasia
  • Immunomodulators such as azathioprine should be introduced early to avoid disease progression, including in those with persistent mild disease and especially in patients with adverse prognostic factors
No therapy / dietary therapy. Consider mesalazine

  • Three-six monthly review reasonable (possibly remotely) with facility for urgent review if symptoms recur
  • Ensure treatments optimized (correct therapy, correct dose, and compliance)
Regular reassessment

 

ECCO Statement 10F (2010)

Neither prednisolone/prednisone [EL5, RG D] nor budesonide [EL1a, RG B] should be used as maintenance treatment in paediatric Crohn's disease.

ECCO Statement 10H (2010)

Azathioprine or mercaptopurine is effective for the maintenance of remission [EL1b, RG A]. Early introduction should be considered at the time of remission induction with either corticosteroids or exclusive enteral nutrition as a part of the treatment regimen in newly diagnosed paediatric patients with severe or extensive Crohn's disease [EL1b, RG A].

ECCO statement 6C (CD 2016)

For patients with extensive disease, thiopurines are recommended for maintenance of remission [EL1]. In patients with aggressive/severe disease course or poor prognostic factors, an anti-TNF-based strategy should be considered [EL5]

Add thiopurine

  • Confirm active disease (consider need to re-evaluate extent). Treatment depends on duration of remission, concurrent therapy, adherence to therapy and patient preference
  • Treat as moderate disease if relapse of mild disease within 3 months
  • Relapse defined as CDAI>150 (HBI>4), with increase in CDAI >70-100 (increase in HBI >/= 3 points)
Relapse

  • Remission for at least 1 year, with normal blood parameters, low faecal calprotectin, and healed mucosa
Long term, deep remission

ECCO Statement 10I (2010)

Methotrexate is effective in maintaining remission in patients resistant or intolerant to azathioprine/mercaptopurine [EL2b, RG B].

Methotrexate

ECCO Statement 10J (2010)

Infliximab is effective for induction of remission in paediatric Crohn's disease patients with moderate to severe disease who are refractory to or intolerant of standard induction therapy [EL2b, RGB]. Regular infliximab infusions can maintain remission for patients with an initial response [EL1b, RGA] and may be effective at closing fistulae [EL4, RGC], although a significant proportion will require dose modification [EL4, RG C].

ECCO statement 6B (CD 2016)

If a patient has a relapse, escalation of the maintenance treatment can be considered to prevent disease progression [EL2]. Steroids should not be used to maintain remission [EL1]. Surgery should always be considered as an option in localized disease [EL4]

ECCO statement 6C (CD 2016)

For patients with extensive disease, thiopurines are recommended for maintenance of remission [EL1]. In patients with aggressive/severe disease course or poor prognostic factors, an anti-TNF-based strategy should be considered [EL5]

Anti-TNF

ECCO statement 6B (CD 2016)

If a patient has a relapse, escalation of the maintenance treatment can be considered to prevent disease progression [EL2]. Steroids should not be used to maintain remission [EL1]. Surgery should always be considered as an option in localized disease [EL4]

Re-start algorithm

  • Paediatric-onset disease often follows a more severe disease course, hence caution advised when considering withdrawal of therapy
Consider stopping therapy

 

ECCO Statement 10K (2010)

Elective surgery should be considered in children with disease resistant to medical therapies, especially in pre-pubertal or early pubertal children with growth failure and localized Crohn's disease [EL4 RGC].

ECCO statement 6B (CD 2016)

If a patient has a relapse, escalation of the maintenance treatment can be considered to prevent disease progression [EL2]. Steroids should not be used to maintain remission [EL1]. Surgery should always be considered as an option in localized disease [EL4]

Further information


Elective surgery

Moderate or severe disease

Moderate or severe disease

ECCO Statement 10C (2010)

Both exclusive enteral nutrition (EEN) and corticosteroids are effective for induction of remission irrespective of disease activity or location [EL1a, RGA]. However, EEN has fewer side effects and promotes growth [EL2b RGB]. Elemental enteral formula is not more effective compared to polymeric formula feeds [EL3, RG C].

Further information

  • As effective as corticosteroids but safer and enhances nutrition
  • Effect independent of disease location
  • No added benefit in providing elemental nutrition
Exclusive enteral nutrition

No response

 

Partial / complete response

ECCO Statement 10D (2010)

Budesonide is effective and favoured over prednisolone in mild to moderate active ileo-caecal CD because of significantly fewer side effects [EL1b, RG A]. The role of budesonide in the treatment of severe or extensive Crohn's disease is uncertain.

ECCO Statement 10H (2010)

Azathioprine or mercaptopurine is effective for the maintenance of remission [EL1b, RG A]. Early introduction should be considered at the time of remission induction with either corticosteroids or exclusive enteral nutrition as a part of the treatment regimen in newly diagnosed paediatric patients with severe or extensive Crohn's disease [EL1b, RG A].

ECCO statement 6C (CD 2016)

For patients with extensive disease, thiopurines are recommended for maintenance of remission [EL1]. In patients with aggressive/severe disease course or poor prognostic factors, an anti-TNF-based strategy should be considered [EL5]

Further information

  • Budesonide as effective as Prednisolone to induce remission in mild to moderate disease (link to definition of mild to moderate)
  • Budesonide has less side effects though can cause adrenal suppression in children after 1 week
  • 15% steroid non-responsive; 30% steroid dependent at 1 year. Do not use steroids as maintenance therapy
Corticosteroids (Budesonide for mild/moderate ileo-caecal disease) and thiopurine

 

ECCO Statement 10F (2010)

Neither prednisolone/prednisone [EL5, RG D] nor budesonide [EL1a, RG B] should be used as maintenance treatment in paediatric Crohn's disease.

ECCO Statement 10H (2010)

Azathioprine or mercaptopurine is effective for the maintenance of remission [EL1b, RG A]. Early introduction should be considered at the time of remission induction with either corticosteroids or exclusive enteral nutrition as a part of the treatment regimen in newly diagnosed paediatric patients with severe or extensive Crohn's disease [EL1b, RG A].

ECCO statement 6C (CD 2016)

For patients with extensive disease, thiopurines are recommended for maintenance of remission [EL1]. In patients with aggressive/severe disease course or poor prognostic factors, an anti-TNF-based strategy should be considered [EL5]

Add thiopurine

No response

Response / remission

Resistance / intolerance

Ongoing relapse

 

  • Reassess 6-12 weekly with biomarkers; with facility for urgent review if symptoms recur
  • Consider objective investigations after 1 year or if symptoms
  • Review management of medically induced remission algorithm
Regular reassessment

ECCO Statement 10I (2010)

Methotrexate is effective in maintaining remission in patients resistant or intolerant to azathioprine/mercaptopurine [EL2b, RG B].

Methotrexate

 

ECCO Statement 10J (2010)

Infliximab is effective for induction of remission in paediatric Crohn's disease patients with moderate to severe disease who are refractory to or intolerant of standard induction therapy [EL2b, RGB]. Regular infliximab infusions can maintain remission for patients with an initial response [EL1b, RGA] and may be effective at closing fistulae [EL4, RGC], although a significant proportion will require dose modification [EL4, RG C].

ECCO statement 6B (CD 2016)

If a patient has a relapse, escalation of the maintenance treatment can be considered to prevent disease progression [EL2]. Steroids should not be used to maintain remission [EL1]. Surgery should always be considered as an option in localized disease [EL4]

ECCO statement 6C (CD 2016)

For patients with extensive disease, thiopurines are recommended for maintenance of remission [EL1]. In patients with aggressive/severe disease course or poor prognostic factors, an anti-TNF-based strategy should be considered [EL5]

Anti-TNF

  • Ensure thiopurine dose and compliance optimized; rectify if necessary, and consider further course of enteral nutrition / corticosteroids rather than anti-TNF if duration of recent remission > 1y
  • Low threshold for anti-TNF in those with adverse prognostic factors
Relapse

Long term, deep remission

ECCO Statement 10J (2010)

Infliximab is effective for induction of remission in paediatric Crohn's disease patients with moderate to severe disease who are refractory to or intolerant of standard induction therapy [EL2b, RGB]. Regular infliximab infusions can maintain remission for patients with an initial response [EL1b, RGA] and may be effective at closing fistulae [EL4, RGC], although a significant proportion will require dose modification [EL4, RG C].

ECCO statement 6B (CD 2016)

If a patient has a relapse, escalation of the maintenance treatment can be considered to prevent disease progression [EL2]. Steroids should not be used to maintain remission [EL1]. Surgery should always be considered as an option in localized disease [EL4]

ECCO statement 6C (CD 2016)

For patients with extensive disease, thiopurines are recommended for maintenance of remission [EL1]. In patients with aggressive/severe disease course or poor prognostic factors, an anti-TNF-based strategy should be considered [EL5]

Anti-TNF

ECCO Statement 10K (2010)

Elective surgery should be considered in children with disease resistant to medical therapies, especially in pre-pubertal or early pubertal children with growth failure and localized Crohn's disease [EL4 RGC].

ECCO statement 6B (CD 2016)

If a patient has a relapse, escalation of the maintenance treatment can be considered to prevent disease progression [EL2]. Steroids should not be used to maintain remission [EL1]. Surgery should always be considered as an option in localized disease [EL4]

Further information


Elective surgery

 

  • Paediatric-onset disease often follows a more severe disease course, hence caution advised when considering withdrawal of therapy
Consider stopping therapy

ECCO Statement 10K (2010)

Elective surgery should be considered in children with disease resistant to medical therapies, especially in pre-pubertal or early pubertal children with growth failure and localized Crohn's disease [EL4 RGC].

ECCO statement 6B (CD 2016)

If a patient has a relapse, escalation of the maintenance treatment can be considered to prevent disease progression [EL2]. Steroids should not be used to maintain remission [EL1]. Surgery should always be considered as an option in localized disease [EL4]

Further information


Elective surgery

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