Enfermedad de Crohn ileocólica. Forma pediátrica severa de debut. Recurrencia precoz tras suspensión de tratamiento biológico
Our patient is a 14-year-old male with no relevant personal or family history, who has had clinical symptoms of abdominal pain for approximately three weeks. The pain was primarily located in the pubic region and in the right iliac fossa (RIF) and worsened on ingestion. He reported increased frequency of bowel movements (BM), varying in the range of 2–4 BM/day, of soft consistency. The patient had lost approximately 12 kg of weight since the symptoms started. In the last 48 hours he developed a fever of up to 38º with a flare-up of the pain, so he was admitted through the Emergency Department.
• Pale skin and mucosa. Temperature: 37.2º.
• Height: 159 cm. Current weight: 36 kg (previous weight 48 kg).
• Cardiopulmonary auscultation (CPA): no abnormalities.
• Diffusely distended abdomen, painful on palpation when empty and in the RIF.
• Blood count: anaemia with haemoglobin (Hb) at 9.8 g/dl and marked iron deficiency. Leukocytes: number and formula within normal ranges.
• Biochemistry: albumin 3.2 g/dl, C-reactive protein (CRP) 54 mg/l (normal range 0-5). The remaining biochemical parameters are within normal ranges.
• Faecal calprotectin: 1,200 mg/g (normal range 0-50 mg/g).
• Coprocultures and faecal parasite study: negative.
• Immunity to hepatitis B virus (HBV) and chicken pox (previous vaccination). IgG (+) for cytomegalovirus (CMV) and Epstein-Barr virus (EBV).
• Saccharomyces cerevisiae antibodies (ASCA): (+); anti-neutrophil cytoplasmic antibodies (ANCA): (-).
• Thiopurine methyltransferase or thiopurine S-methyltransferase study (TPMT): 19.2 U/ml (normal TPMT enzyme activity).
• Mantoux test: negative.
• Chest X-ray: no pathological abnormalities.
With the suspected diagnosis of the debut outbreak of inflammatory bowel disease, we performed endoscopic examinations:
COLONOSCOPY (Video 1)
We performed the examination under deep sedation with good tolerance and sufficient preparation.
The anal and rectal digital examinations found no notable results. A full colonoscopy was performed, finding no mucosal pathology in the rectum or sigmoid. Starting from the left colon, we observed ulcerated lesions with serpiginous borders and some depth, with an initial size varying between 0.5 and 0.8 cm, appearing as skip lesions leaving wide areas of preserved interlesional mucosa. Continuing the examination towards the right colon, the ulcers increased in size and depth, joining together to form filled areas with extensive regions of ulceration and denuded mucosa, leading to partial inflammatory stenosis close to the caecal pole, which was somewhat difficult for the colonoscope to pass.
The perivalvular area is affected, with significant inflammatory involvement and ulcerated regions, with no penetration of the ileal loop.
Biopsies were taken for histological study, observing: ulceration and acute and chronic inflammation compatible with chronic inflammatory bowel disease (CIBD), specifically Crohn’s disease, with severe activity (Fig. 1).
The endoscopic findings are suggestive of Crohn’s disease, with moderate-severe involvement of the right colon and probable ileal involvement. Inflammatory-stenotic phenotype.
We performed a magnetic resonance enterography (MR enterography) study to evaluate the involvement, extension and phenotype of the debut in the small intestine (Fig. 2 and Video 2).
MAGNETIC RESONANCE ENTEROGRAPHY STUDY
A MR enterography study was performed following ingestion of a polyethylene glycol solution and the intravenous administration
of buscapina and gadolinium.
The examination was somewhat limited due to the low ingestion of oral contrast (+/- 500 cc).
We observed a notable thickening (approximately 7 mm) in the wall of the last 20 cm of the terminal ileus, as well as in the caecum and practically the entire ascending colon, associated with the rigidity of the ileum and mucosal ulcers. Following the intravenous administration of gadolinium, there an enhancement of the affected areas. The findings are compatible with ileocaecal inflammatory pathology, probably Crohn’s disease, with signs of moderate-high activity.
After performing the tests, the diagnosis of Crohn’s disease with ileocolic involvement (Montreal classification A1L3B2) was confirmed.
Treatment with intravenous corticosteroids was started with no significant clinical improvement, as the patient continued to have mild fever, abdominal pain and frequent bowel movements. As this was a severe debut with important clinical, analytical, endoscopic and radiological involvement and no clear response to corticosteroid treatment, we decided to begin combined induction treatment with infliximab (5 mg/kg in weeks 0, 2 and 6) and azathioprine (2.5 mg/kg). The patient showed very good response to the treatment with disappearance of symptoms, recovery of weight and early normalisation of analytical parameters.
The combined treatment was continued for one year, during which the patient remained in clinical remission with consistently normal inflammatory activity parameters (CRP and calprotectin), so we decided to perform a repeat endoscopy and MR enterography to assess the situation and to decide on the approach for maintenance treatment.
REPEAT EXAMINATIONS IN PATIENT UNDER COMBINED TREATMENT, MAINTAINING CLINICAL REMISSION
Annual repeat colonoscopy
We performed a colonoscopy under deep sedation with good tolerance and sufficient preparation (Video 3).
The anal and rectal digital examinations found no notable results.
A full colonoscopy was performed, observing pseudodiverticular scarring lesions with fibrous tracts and areas of contracture in the ascending colon, which previously had the most inflammatory involvement. We found no ulcerative lesions or lesions suggestive of activity, and we found only chronic changes relating to previous inflammatory activity. The ileocaecal valve could not be clearly identified to penetrate the ileum, due to scar contractures in this area.
Annual repeat magnetic resonance enterography
The study was performed following the ingestion of a polyethylene glycol solution and the intravenous administration of buscapina and gadolinium (Video 4 and Fig. 3).
Progress was very favourable compared to the previous examination, and we found no signs of involvement of the terminal ileus at this time. Thickening of the caecal wall, probably related to previous activity, with no signs of inflammatory activity.
Given the patient’s current condition: in clinical-analytic remission since the start of treatment, with signs of mucosal scarring and radiological inactivity, as this was a debut outbreak in a patient naive to azathioprine treatment, we decided to withdraw the infliximab and continue the maintenance treatment with azathioprine in monotherapy. The patient remained asymptomatic for 6 months.
EARLY CLINICAL RECURRENCE FOLLOWING WITHDRAWAL OF INFLIXIMAB
Six months after the withdrawal of the infliximab treatment, the patient experienced clinical recurrence with diarrhoea, abdominal pain and weight loss, requiring steroid treatment.
A new colonoscopy and a MR enterography were performed to evaluate the current situation.
Colonoscopy following clinical recurrence (Video 5)
A colonoscopy was performed, during which
we observed a severe recurrence of the disease with similar involvement in terms of shape, extent and location to those of the debut colonoscopy.
We observed ulcerative lesions of a star-shaped, patchy morphology and alternating depth with areas of preserved mucosa in the left colon and at the start of the transverse colon. Continuing towards the right colon, the ulcers increased in size and we observed severe involvement with a cobbled appearance in the ascending colon, with inflammatory stenosis close to the caecal region, which was difficult for the paediatric colonoscope to pass. There was patchy involvement of the caecal region and the valvular region could not be correctly identified for penetration of the ileum.
Magnetic resonance enterography following clinical recurrence
The MR enterography study was performed following the ingestion of a polyethylene glycol solution and the intravenous administration of buscapina and gadolinium (Video 6, Fig. 4 and Fig. 5).
We observed notable involvement of the caecum and ascending colon (in conical form), with wall thickening in approximately the last 10 cm of the ileum and signs of submucosal oedema. All lesions showed notable restriction of diffusion. There are isolated locoregional adenopathies.
These findings suggest recurrence in the referenced locations, with signs of moderate-high activity.
Given the clinical involvement and the tests performed, the infliximab treatment was reintroduced, carrying out a new induction regimen and maintaining the treatment as a combined regimen. The patient again responded to the drug, reaching clinical-analytical remission with normalised CRP and calprotectin after 4 months, currently remaining in clinical remission.
SUPPORTING SCIENTIFIC UPDATE
• II.1.1.1. Endoscopy in the diagnosis of Crohn’s disease
• II.1.1.2. Endoscopy in the follow-up of Crohn’s disease
• II.1.3. Endoscopy in paediatric inflammatory bowel disease
• II.2.2.1. Enterography by computed tomography and magnetic resonance imaging
• II.6. Seeking consensus between the different diagnostic and follow-up options for inflammatory bowel disease. Interpretation of the results obtained