Crohn's disease (CD) is an inflammatory disorder characterized by periods of variable clinical activity. There is an increasing incidence of CD over last two decades. 15-25% of all IBD has onset in childhood and two third of this is before 12 years of age.
Presentation:
Typical:
- Pain in abdomen
- Diarrhea + blood in stools
- Weight loss
- Early satiety
- Oral ulceration
Atypical:
- Growth failure
- Anorexia, malaise
- Pyrexia of unknown origin
- Osteoporosis
- .Delayed puberty
- Dermatologic-E nodosum, p yoderma gangrenosum, Oral/facial granulomatosis
Disease phenotypes:
Disease location
- Colon
- Ileo-colonic
- Small bowel only
- Gastro duodenal
- Extraintestinal
Biological behavior
- Colon
- Inflammatory
- Penetrating
- Stenosing
Family history of IBD increases the risk of developing IBD 4-20 times. There is an absolute risk of 7% of IBD in first degree relative.
Investigations:
Screening :
- Decreased Hb
- Increased ESR
- Increased platelets
- Increased CRP
- Decreased albumin
- Stool-occult blood/lactoferrin/calprotectin
Confirmatory:
Derology pANCA/ASCA (IgA/IgG)
ASCA-70% subjects with Crohn's are positive. High titres predict early onset and fibrostenosing/penetrating disease pANCA-positive in 20% subjects, suggests colonic disease Anti OmpC
- UGI endoscopy – Focal gastritis ~ 50-60%, Granuloma ~ 28-60%
- Colonoscopy and biopsy – for disease extent, activity and histological confirmation
- For small bowel disease
- Capsule endoscopy
- Barium meal follow through
- Tc99m HMPO leucocyte scan
- Indium labeled WBC scan
- CT/USG for extraintestinal extension.
Grading;
- Pediatric Crohn's disease activity index (PCDAI) – determined by historical/lab/physical examination.
- History: pain, number/consistency of stools, patient functioning.
- Physical examination: abdominal mass/tenderness, perirectal disease.
- Laboratory: Hct, Albumin, ESR, Wt gain/loss, Height velocity.
- A score may range from 0-100, with 100 showing maximum activity.
Modified PCDAI:
- Detects short term changes in disease activity and strong correlation with PCDAI
- No need for lab investigations as it is based only on history and physical examination (history: abdominal pain/stool/patient functioning; physical examination: Weight/abdominal/perirectal disease).
- A score of 0 means no activity ad a score of 70 means maximum disease activity
Genetic association in CD:
- On chromosome 16 NOD2/CARD 15 (Caspase activation and recruitment domain) gene has been identified recently. It is expressed in macrophages and involved in recognition of bacterial lipopolysaccharides and thus regulation of NF-kB activation. Homozygotes have 20 fold risk of CD.
- NOD2 positivity is associated with ileal/structuring disease and earlier disease onset. No relation with response to infliximab.
Therapy :
Goals-short term
- Control symptoms
- Induce remission
long term
- prevent relapse
- reduce hospitalization/surgery
- Reduce complications
- Restore normal growth
Active disease :
Mild moderate:
- 5ASA prep/antibiotics-metronidazole/ciprofloxin
- Oral steroids prednisone/budesonide
- Enteral nutrition
Moderate severe
- Parenteral steroids
- Early use of immunosuppressants (azathioprine / 6 Mercaptopurine)
Methotrexate
- Anti TNF alpha antibody (Infliximab)
- Maintenance
- No role of steroids
- Mesalamine?
- 6MP/Azathioprine
- Methotrexate
- Infliximab
- Surgery: Finally needed by approx 50% subjects
- Indicated in- Complications-stricture/abscess
- Disease resistance to maximal treatment
- Nutrition supplementation: very important
- Deficiencies of the following nutrients are common and should be treated-
- Iron due to blood loss
- Vitamin B12 in ileal disease
- Folate due to treatment with mesalamine/methotrexate
- Zn-malabsorption/decreased intake
- Ca, Vitamin D – malabsorption and treatment with steroids
- Psychosocial support
- Crucial for overall functioning of the child
- Support of Crohn's societies for both child/parent
- Encourage normalcy in life.
Conclusions:
Recent report of CD from India
High index of suspicion for timely diagnosis and prevention of complications
No single ideal therapy; therapy should be balanced with monitoring for side effects
Regular follow-up is a must for success.
