GUIDELINE ON THE INVESTIGATION & MANAGEMENT OF
PAEDIATRIC INFLAMMATORY BOWEL DISEASE
(Please note that each patient needs a treatment plan negotiated with the patient and
family by the Paediatric Gastroenterologist and Team)
INTRODUCTION
Inflammatory Bowel Disease ( IBD) encompasses two related but distinct disorders of as
yet unknown cause. Current opinion favours the hypothesis that IBD results from an
interaction between immunological, genetic and environmental factors. Many believe the
incidence has increased substantially in recent years – 2.2 to 6.8/100 000 in
paediatric/adolescent population worldwide. About 25% of IBD onset is in the paediatric
age range particularly in adolescence but with 5% occurring before age10. Genetic
susceptibility is strongly implicated in the pathogenesis of IBD- 30% of children have a
positive family history and both diseases can exist in the same family.
Current therapeutic goals in children and adolescents are to diagnose/ treat relapses early,
improve clinical management and reduce morbidity. Early effective treatment of relapse
prevents prolonged episodes.
The important issues in this age group are –
Growth and Puberty Development
Bone mineralisation
Psychosocial issues and coping with chronic illness
Educational attainment
Transitional care to Adult Gastroenterology
There is a significant correlation between symptom duration and the degree of growth
impairment present.
Colorectal cancer occurring in adulthood is a recognised complication of longstanding
colonic involvement ( chronic bowel inflammation).
There is a lot of ongoing research in IBD looking at mucosal immunity, gut flora and use of
probiotics, infective agents, diet, fish oils, passive smoking, genetics etc. It is hoped that
this will lead to better and more tailored treatments. The choice of drug therapies for an
individual patient may be rationalised on the basis of genotype e.g use of thiopurine
methyltransferase(TPMT) genotyping or phenotyping in patients receiving azathioprine;
NOD2/ CARD 15 genotyping of patients requiring infliximab for refractory crohn’s, etc.
ULCERATIVE COLITIS
PATHOLOGY
Ulcerative Colitis is a chronic and diffuse inflammation of the mucosa and submucosa.
Endoscopically abnormal changes are seen in rectum and distal colon but involvement of the
whole colon i.e Pancolitis is the most common form (62%). There may be an inflammatory
reaction in the distal ileum – so called backwash ileitis. Macroscopically
there may be erythema, oedema, friability with bleeding, granularity and erosions and
pseudopolyps. The characteristic histological features, in brief, are acute and chronic
inflammatory cell infiltrate in the lamina propria, distortion of crypt architecture, presence of
crypt abscesses and goblet cell depletion.
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CLINICAL FEATURES
o
o
o
o
o
o
o
o
o
Diarrhoea, often with blood and mucus
Lower abdominal pain relieved on defecation, sometimes backache
Tenesmus
Urgency with fecal incontinence
Abdominal tenderness
Maybe fever, anemia and hypoalbuminemia
Extraintestinal features – joint pains, rash, liver disease etc
Growth retardation is less common
Occasionally constipation occurs in proctitis
Can be differentiated into mild, moderate and severe –
GLOBAL DISEASE ACTIVITY IN PEDIATRIC IBD
DIARRHEA
BLOOD PR
ABDO. PAIN
WEIGHT LOSS
FEVER
ESR
HEMOGLOBIN
ALBUMIN
MILD
0-6/ day
occasional
mild
none
no
normal
normal
normal
MODERATE
> 6/ day
often
moderate
1kg or less
no
<30mm/hr
>6.5 mmol/L
>30g/L
FULMINANT
> 10/ day
massive
severe
>1kg
>37.5 C
>30mm/hr
< 6.5mmol/L
< 30g/L
Disease activity is determined by signs, symptoms and laboratory parameters. Abdominal
pain may override the above
INVESTIGATIONS
FBC
ESR
Inflammatory markers (not universally
CRP
elevated )
A1 acid glycoprotein
Albumin
LFTs
U&E
pANCA (?ASCA) and autoimmune screen ( Approx. 60% of U.C have a circulating
autoantibody perinuclear antinuclear cytoplasmic antibody, pANCA and about 60% of
cases of crohn’s disease have anti- saccharomyces cerevesiae antibody, ASCA but neither
is sufficiently sensitive or specific for routine diagnostic use.)
Stool mc&s, ova, cysts and parasites
Endoscopic examination of the whole colon with biopsies is required to assess severity and
extent of the inflammation and to confirm the diagnosis histologically.
TREATMENT
Mild disease is treated with sulfasalazine (max 60mg/kg/day) See BNF for Children –
Treatment and maintenance doses ( 2-4 divided doses) Side effects more common with
sulfasalazine than mesalazine and dose related – headache, nausea, epigastric pain,
diarrhoea
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If distal colitis / proctitis present - additional topical treatment in form of steroid enema or
sulfasalazine suppositories or enema.
Moderate disease – oral steroids added ( soluble prednisolone 1-2mg/kg/day max 40mg)
Gradually reduced after 1-2 weeks when clinical condition improving and then stepwise
down by 5mg/week to 5mg daily for 7 days to 5mg alternate days for 7 days, then stop.
Occasionally alternate day steroids required for longer.
Moderate to Severe disease – admit for IV hydrocortisone and observation
Antispasmodics and agents which decrease gut motility should not be given as may
precipitate development of toxic megacolon.
Severe, fulminating colitis is a medical emergency –
Nil by mouth
IV fluids
? Rehydration, blood transfusion, albumin infusion
IV Hydrocortisone
IV broad spectrum antibiotics (penicillin, gentamicin and metronidazole)
If malnourished, parenteral nutrition may be required.
Many children with severe colitis will respond to aggressive medical therapy within 7 –10
days but if no improvement or complication arises – toxic megacolon, colonic
haemorrhage or perforation then surgery is required.
Maintenance of remission – If requiring frequent steroid courses (relapse within 6
months) or steroid dependent then immunosuppressants such as Azathioprine or
cyclosporin may be indicated. Azathioprine can show clinical improvement after 6 weeks
but can take 3 months to have maximum effect. The dose is usually built up slowly - Please
see our Shared Care Guideline for Azathioprine. We are now doing the TPMT test ( 2 mls
blood in EDTA bottle to Clinical Chemistry) before starting Azathioprine or if already
started before increasing dose above 1mg/kg.
PROGNOSIS
90% of children and adolescents will experience one or more relapses after initial treatment
and 30% require a colectomy at some point.
INDETERMINATE COLITIS
This term is reserved for cases of colitis in which findings are not sufficient to allow
differentiation between crohn’s colitis or ulcerative colitis. This occurs in 10%. It may be
considered as a diagnosis in its own right.
CROHN’S DISEASE
PATHOLOGY
Crohn’s is a patchy transmural inflammation which may involve any part of the GI Tract
from lips to anus but typically with rectal sparing. Terminal ileitis is common with variable
involvement of the colon 50 – 70% (ileocolic disease). Fistulae and strictures may occur.
Macroscopically there may be ulcerations, fissures and cobblestoning. Microscopically the
findings are, in brief, cryptitis or crypt abscesses, distortion of crypt architecture, presence
of fibrosis and histiocytic proliferation and the characteristic noncaseating granulomas.
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CLINICAL FEATURES
Usually insidious onset and symptoms and signs can be subtle. The manifestations of the
disease depend on the site of involvement. Common presentations are –
o
o
o
o
o
o
o
o
o
o
Periumbilical colicky abdominal pain
Diarrhoea with or without blood
Loss of appetite, nausea , lethargy
Growth failure
Mouth ulcers
Lip Swelling
Perianal tags, fissures, fistulae, abscess
Growth failure / delayed puberty
Abdominal tenderness – generalised/ localised
Abdominal mass (transmural inflammation causes loops of bowel to be
matted together) , palpable thickened bowel
o Extraintestinal features – (more common with colonic disease)
arthralgia/ arthritis ( joint involvement most common)
uveitis
clubbing
erythema nodosum, pyoderma gangrenosum
sacroileitis/ ankylosing spondylitis
liver dysfunction, sclerosing cholangitis
sometimes pancreatitis
rarely oxalate renal stones
rarely hypercoagulable states
BEWARE can mimic anorexia nervosa particularly where weight loss is predominant.
Chronic diarrhoea in Crohn’s can sometimes be due to mucosal dysfunction, bile acid
malabsorption or bacterial overgrowth.
INVESTIGATIONS
Same as above but also check for specific nutrient deficiencies e.g iron, B12 & folate. Also
including upper and lower GI endoscopy with biopsies, Abdominal ultrasound with Barium
Meal and Follow through to look for small bowel disease. On Ultrasound may see bowel
thickening (chronic inflammation & oedema), mesentery thickening and mesenteric lymph
node enlargement. Luminal narrowing and strictures may be seen in Barium Study.
If isolated terminal ileitis check yersinia titres, consider mantoux test (if possible TB).
TREATMENT
The medical treatment should be individualised based on site of intestine involved/
severity/ extraintestinal manifestations and nutritional status.
Nutritional Therapy – Modulen IBD, perative, elemental diet result in comparable
remission rates to steroids and allow bowel rest, reduce intestinal permeability, improved
nutrition and is particularly useful in upper GI Crohn’s. May need to be given via NG tube.
Can also be used as supplementary feeding. Paediatric Dietetic advice should be sought.
Steroids – induce remission in over 70% of patients. Use soluble prednisolone 12mg/kg/day max 40mg ( Monitor for side effects e.g B.P, Urinalysis and suspect Benign
Intracranial Hypertension if headaches which can develop particularly if on prolonged
course)
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Aminosalicylates ( released by bacterial enzymes in colon and acts on epithelial cells in
gut lumen) – can be useful for Crohn’s colitis. There are a variety of sustained release
formulations to deliver mesalazine to the site of inflammation at the distal ileal or colonic
mucosa, thereby minimising systemic absorption.
Mesalazine (30-60mg/kg/day) Usually in form of Asacol max.800mg tds or Pentasa 1g tds
for treatment dose ; sulphasalazine (max 60mg/kg/day) usually in the form of Salazopyrin
max. 1g tds/qds for treatment dose until remission occurs. Asacol is a delayed release form
of mesalazine which depends on appropriate intestinal pH and Pentasa releases mesalazine
continuously over a period of several hours.
Antibiotics – metronidazole (7.5mg/kg max 400mg x 3 times a day for 14 days) or
ciprofloxacin (7.5mg/kg x 2 times a day for 14 days)( max 500mg b.d)orally used in
perianal disease. Sometimes surgery is required for perianal abscess or fistulae (pelvic MRI
may be required)
Immunosuppressants – Azathioprine (1-3mg/kg/day) used to maintain remission in
moderate to severe disease (steroid sparing effect). Do TMPT levels ( this will only identify
¼ of those who subsequently develop bone marrow suppression)
(separate protocol available as requires close monitoring for side effects)
Other medical therapies – include cyclosporin, methotrexate, infliximab (antiTNF
monoclonal antibody)
Surgical Intervention – for intestinal obstruction due to stricturing disease, fistula
formation, haemorrhage and perforation and for failure of medical treatment (if limited
disease) particularly if growth failure.
 When inflammation extends to serosal surface perforation or intraabdominal
abscess may occur – increasing abdominal pain, fevers, tenderness to palpation or
peritoneal signs should prompt urgent evaluation for this complication.
PROGNOSIS
Patients with colonic disease suffer more extraintestinal complications and more operations
than those with small bowel disease. A third of children continue to have mild GI
symptoms and two-thirds will vary between severe exacerbations and remissions. 17%
require surgery but there is a 50-80% relapse rate within 3 years of surgery.
HARVEY-BRADSHAW INDEX of Crohn’s Disease Activity
A
General well-being
B
Abdominal pain
C
Number of liquid stools per day
D
Abdominal mass
0 = very well
1 = slightly below par
2 = poor
3 = very poor
4 = terrible
0 = none
1 = mild
2 = moderate
3 = severe
0 = none
1 = dubious
2 = definite
3 = definite and tender
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E
Complications
Score 1 for each of arthralgia, uveitis,
erythema nodosum, pyoderma
gangrenosum, aphthous ulcers, anal
fissure, new fistula, abscess
The patient scores these items for the previous day as opposed to the more complex
systems used over a seven day period This index can be used to assess response to
treatment and monitor long term progress. A score 8/9 indicates severe disease.
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ULCERATIVE COLITIS
Table 1
* Use Ciclosporin
Guideline with Pharmacy Advice
Mild or localised
disease
Mesalazine or
Sulphasalazine in 2 –4
divided doses
Consider if distal
Hydrocortisone enema or
Mesalazine or sulphasalazine
enema/ suppositories b.d for 2-4
weeks then o.d for 2-4 weeks
weeks
Consider low residue diet
Moderate Disease
Oral Prednisolone (soluble)
1-2mg/kg max 40mg
If unwell
Admit for IV Hydrocortisone
and observation
+
Build up Mesalazine/
sulphasalazine to therapeutic
doses
Remission
Maintenance dose regime
Severe Disease
Surgical Consultation
Nil by mouth
PFA for ? Toxic Megacolon
? Broad spectrum antibiotics
IV Hydrocortisone
Refractory disease *IV Ciclosporin/ Azathioprine
Check for
 Dehydration
 Electrolyte disturbance
 Anemia
 Hypoalbuminemia
Surgical Resection if
failure to respond
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CROHNS DISEASE
Table 2
Acute Exacerbation
Elemental or polymeric liquid
diet for 6-8 weeks- graded
return to normal diet
Commenced by dietician
May need NG Tube
Not responding after 2 weeks
Add Mesalazine
preparation if
colonic disease
Oral Prednisolone (soluble)
1-2mg/kg max 40mg
Not responding
IV Hydrocortisone
50 – 100 mg x 4 times/day
Oral Steroids, on
reducing course as
clinically improves
Refractory Disease
Azathioprine
Refractory
Maintenance liquid diet
for 1 year post diagnosis
and continue mesalazine
preparation
Consider if
treatment fails
*Infliximab
particularly if
fistula and/or
perianal disease.
others – bleeding
from ileocolonic
anastomosis or
bleeding ileal ulcer
Surgery
* See NICE Guidance
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Table 3
Perianal Crohn’s Disease
Orogranulomatous
Disease
May already be
on immunomodulating
drugs
Elimination Diet ( Seek
Dietetic advice)
Metronidazole
and/or Ciprofloxacin
( Also consider
elemental diet)
Not Responding
Not responding
Trial of Tacrolimus
Ointment 0.03%
May need
surgical
intervention
Topical steroids e.g
Adcortyl paste /
steroid cream
Trial of Tacrolimus
ointment
Not responding
Consider Azathioprine
May need MaxilloFacial opinion re. lip
steroid injection
Not responding
Infliximab particularly
if fistula
(administered under
specialist supervision)
Consider oral immuno
suppression
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REFERENCES
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Inflammatory Bowel Disease in Children and Adolescents: Working Group Report
of the First World Congress of Paediatric Gastroenterology, Hepatology and
Nutrition. Journal of Pediatric Gastroenterology and Nutrition: Volume 35
Supplement 2 August 2002 ppS151-S158
Current Diagnosis, Management and Morbidity in Pediatric Inflammatory Bowel
Disease: Acta Paediatrica 2001Apr: 90(4): 400-5
Textbook of Paediatrics. Harcourt: Forfar and Arneils fifth edition
Inflammatory Bowel Disease in Pediatric and adolescent patients: clinical,
therapeutic and psychosocial considerations: Gastroenterology 2004 May:126
(6):1550-60
Essential Pediatric Gastroenterology, Hepatology and Nutrition : Stefano
Guandalini 2005 : Mc Graw- Hill Company.
Unravelling the complex genetics of inflammatory bowel disease , RK Russell, DC
Wilson and J Satsang: Archives of Disease in Childhood 2004; 89:598-603
Pediatric Gastrointestinal Disease, third edition: Walker, Durie, Hamilton, WalkerSmith, Watkins
Shared Care Azathioprine Protocol, Paediatric Gastroenterology, QMC
Adult Ciclosporin for Fulminent Colitis Guideline, QMC
Lancet 1980;i:514 A Simple Index of Crohn’s Disease Activity, Harvey RF,
Bradshaw JM.
Buller HA, Maas SM. Consensus inflammatory intestinal diseases in children:
ulcerative colitis and crohn disease. Workgroup Academic Medical Centre.
Amsterdam 1996
Management of inflammatory bowel disease . Chapter 20. Manu Nayar and
Jonathon Rhodes. Gastroenterology Update
*
Dr Mary Weston
Dr Charlie Charlton
June 06
Date of Review: June 08
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