CROHN'S DISEASE: FROM SUSPICION TO SILENCE
Dr. Anshu Srivastava *
Assistant Professor, Department of Pediatrics King George's Medical University, Lucknow *
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:
  1. Pain in abdomen
  2. Diarrhea + blood in stools
  3. Weight loss
  4. Early satiety
  5. Oral ulceration

Atypical:
  • Growth failure
  • Anorexia, malaise
  • Pyrexia of unknown origin
  • Osteoporosis
  • Delayed puberty
  • Dermatologic: E-nodosum, pyoderma 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:

Serology 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.
  1. UGI endoscopy - Focal gastritis ~ 50-60%, Granuloma ~ 28-60%
  2. Colonoscopy and biopsy - for disease extent, activity and histological confirmation
  3. For small bowel disease
    • Capsule endoscopy
    • Barium meal follow through
    • Tc99m HMPO leucocyte scan
    • Indium labeled WBC scan
  4. 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

Goals: Long term
  • prevent relapse
  • reduce hospitalization/surgery
  • Reduce complications
  • Restore normal growth

Active disease:
Mild-moderate:
  • 5ASA prep/antibiotics-metronidazole/ciprofloxacin
  • 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.
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