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ESPGHAN IBD Diagnostic Practice Recommendations -Revised Porto Criteria

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Recently ESPGHAN assembled an international group of European experts in pediatric inflammatory bowel disease (PIBD) to establish practice recommendations (JPGN 2014; 58: 795-806).  Their aim was “to revise the original Porto criteria using an evidence-based approach and consensus process to yield specific practice recommendations for the diagnosis of PIBD.”

Before detailing some of their recommendations, I want to state my main criticism: these recommendations do not consider cost or cost-effectiveness. This is important since we do not live in a world where costs are irrelevant.

Some specific recommendations/observations:

1. “We recommend performing small bowel imaging in all suspected cases of IBD at diagnosis; this may be deferred in typical UC.”  In addition, all suspected cases of IBD should undergo esophagogastroduodenoscopy (EGD) and ileocolonoscopy.  “The diagnostic yield of an EGD to diagnose Crohn’s disease (CD) in patients with an otherwise normal workup [ileocolonoscopy/small bowel imaging] is ~7.5%. ”

2. The authors clarify the use of IBD-unclassified (IBD-U). “IBD-U should be …for patients with colitis and highly atypical findings.” Atypical findings for ulcerative colitis: include rectal sparing, and cecal patch (present in 2% of pediatric patients with left-sided colitis).  Table 3 suggests that if at least one “class 2” (rare feature) exists or at least 2 “class 3” (uncommon) feature exists, then labeling IBD-U is appropriate.

Rare (Class 2):

  • significant growth delay
  • histologic and gross sparing of rectum
  • transmural inflammation in the absence of severe colitis
  • duodenal or esophageal ulcers (not due to other causes)
  • multiple aphthous ulcerations in the stomach (not due to other causes)
  • positive ASCA in the presence of negative pANCA
  • mucosal inflammation more severe in proximal colon

Uncommon (Class 3):

  • severe scalloping of stomach or duodenum (not due to other causes)
  • focal chronic duodenitis (not due to other causes)
  • aphthous ulcerations in the colon

3. Crohn’s disease, according to Table 3, should be diagnosed with any of the following:

  • well-formed granulomas anywhere in the GI tract, remote from a ruptured crypt
  • deep serpentine ulcerations, cobblestoning or stenosis anywhere in the small bowel
  • fistulizing disease
  • ileal inflammation in the presence of normal cecum

4. “Normal blood tests do not exclude the diagnosis of IBD”… Fecal markers (eg. calprotectin) are “extremely sensitive in the detection of mucosal inflammation but are not specific for IBD.”

5.”Although small bowel imaging is encouraged in all of the patients with suspected IBD, it is essential in pediatric patients with CD, IBD-U, or atypical UC.”  Magnetic resonance enterography (MRE) is currently the imaging modality of choice in PIBD.  Wireless capsule endoscopy (WCE) is a “useful alternative.”  The authors advocate for imaging because it may “detect small intestinal involvement…and identify disease complications.”

6. Evaluation for primary immune deficiency should be performed in all cases of PIBD  diagnosed <2 years of age.

While the authors acknowledge that “clinical considerations may require taking a course of action that varies from these criteria,” nevertheless, they are likely to influence clinical practice.  My personal belief is that there are many situations in which small bowel imaging will not result in changes in clinical care.  Furthermore, many patients, especially younger patients, would require anesthesia in order to complete a MRE which is an added burden.  In addition, with the added emphasis on assessing response to therapy, one could envision that some patients would be better served with imaging after implementing treatment.

Related blog posts:

 

 

 

Superiority of Anti-TNF Therapy in Children

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This study’s conclusion comes as no surprise:

“In children newly diagnosed with comparably severe CD, early monotherapy with anti-TNFα produced better overall clinical and growth outcomes at 1 year than early monotherapy with an immunomodulator. Further data will be required to best identify children most likely to benefit from early treatment with anti-TNFα therapy.”

Here’s the reference:

Gastroenterology Volume 146, Issue 2 , Pages 383-391, February 2014

Here’s a link to the full text article:  Increased Effectiveness of Early Therapy with Anti-Tumor Necrosis Factor-α Versus an Immunomodulator in Children with Crohn’s Disease

Methods: “From 2008 through 2012 at 28 pediatric gastroenterology centers in North America. Patients were managed by physician dictate. From 552 children (median age, 11.8 y; 61% male; 63% with pediatric CD activity index scores >30; and median C-reactive protein level 5.6-fold the upper limit of normal), we used propensity score methodology to identify 68 triads of patients matched for baseline characteristics who were treated with early anti-TNFα therapy, early immunomodulator, or no early immunotherapy.”

Another reference/link from same issue:

Accuracy of Magnetic Resonance Enterography in Assessing Response to Therapy and Mucosal Healing in Patients with Crohn’s Disease

CCFA IBD Update -Conference Notes (part 2)

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As noted in previous blog post, I wanted to share some notes from recent Atlanta CCFA talk.

The fourth lecture by Jeffry Katz discussed optimizing biologic therapy.  Overall this was an excellent review.  He discussed his general preference for combination therapy since the publication of the SONIC study. Also, he highlighted a smaller study that showed better efficacy with combination therapy in ulcerative colitis as well (DDW 2011, Abstract #835).

With regard to withdrawal of therapy when doing well on combination treatment, he indicated that he sometimes reduces (or stops) dosage of immunomodulator after 1 year but tries to avoid stopping anti-TNF agents.  Relapse rates after stopping infliximab in Crohn’s disease are approximately 50% at 1 year and 75% at 5 years.

His talk reviewed antibodies to infliximab and low therapeutic levels. This has been discussed on this blog previously:

He reviewed risks of the IBD medications.  With regard to psoriasis reactions, he stated that developing skin lesions occur in about 5% and this necessitates drug withdrawal in 1%.  As these skin reactions are often a ‘class effect,’ use of an alternative may be needed.  He stated that he had used ustekinumab in this setting (“but this entails a fight with the insurance company”).

The 5th talk by Doug Wolf reviewed pregnancy in IBD.  Much of the information has also been discussed in this blog recently: Anti-TNFs and Pregnancy | gutsandgrowth

His key points:

  • Probably stop infliximab at gestational week 32
  • Likely give adalimumab up until week 34-36
  • If patient in remission, consider stopping stopping drugs earlier
  • In PIANO registry (n=1000), use of anti-TNFs and immunomodulators was not associated with any complication, including prematurity, spontaneous abortion, intrauterine growth retardation or specific birth defects.  However, there was a significant increase in infant infections up to 12 months of life in the combination therapy group.
  • No live virus vaccines (eg. rotavirus) for first 6 months for infants exposed to infliximab

The last talk that I attended was a pediatric case presentation from Cary Sauer. He presented a teenage boy who had mild disease based on bloodwork and endoscopy who had more severe and extensive disease on magnetic resonance enterography (MRE) (More imaging needed? | gutsandgrowth) and video capsule endoscopy.  He argued that small bowel assessment is worthwhile in every patient at the time of diagnosis as more severe findings could influence the choice to start with top-down therapy.

The final aspect worth mentioning were some of the patient-related information:

1. A pediatric, adolescent, and parent support group will have its first meeting April 23rd 6-7:30 pm at Scottish Rite Children’s Hospital (Main auditorium).  Followup meetings are scheduled for August 27, and October 22. All meetings are free.  Contact CCFA mball@ccfa.org or 646-623-4869 (cell) for more information.

2. CCFA also has “Power of Two.”  This contacts patients/parents with peer mentors.  Interested patients can contact mball@ccfa.org or 404-982-0616.

Magnetic resonance enterography for Crohn’s disease

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Magnetic resonance enterography (MRE) has increasingly been recognized as an effective way to characterize small bowel disease in Crohn’s disease (Inflamm Bowel Dis 2012; 18: 520-28).

In this retrospective pediatric study, 95 patients with Crohn’s disease underwent MRE from 2006-2009.  As expected, terminal ileal disease was the most common site of involvement with 54%; this was followed by ileum with 19%, and jejunum with 17%.  Other findings included solitary jejunal inflammation (3.7%), small bowel stenosis (1.9%) fistula (1%), and abscess (1%).  Specific evidence of inflammation included contrast enhancement, bowel wall thickening or derangement of bowel wall shape.  The images in the article are excellent.

The main advantage of MRE over CT enterography (CTE) is the lack of ionizing radiation.  In addition, MRE can better detect soft tissue contrast suggestive of bowel wall edema.  This helps distinguish acute from chronic inflammation.  Obtaining an MRE is technically more challenging and more costly.  The youngest patient in the study was seven; though the authors note that the youngest patient they have performed MRE was six.

Additional references:

  • More imaging needed?
  • -JPGN 2010; 51: 603.  MRE for suspected IBD.  Useful in pediatric Crohn’s disease.
  • -IBD 2009; 15: 1635. U/S compared favorably with endoscopy in correlating postoperative recurrence with Crohn’s.
  • -Clin Gastro Hepatol 2006; 3: 1221. MRI as accurate in evaluating ileocolonic disease with flareups as endoscopy.
  • -IBD 2004; 10: 452-61. U/S was very helpful in identifying disease and complications.