ECCO CONFER Cases

 

COllaborative Network For Exceptionally Rare case reports  

 

CONFER Cases Round 10 - Call for Similar Cases

ECCO CONFER is now opening the call for similar rare cases for the tenth Round of ECCO CONFER Cases.

Case 1: 

Perianal Crohn's fistula in the absence of luminal disease - natural history, time to progression to luminal disease and management in contemporary clinical practice

Case 2:

Exposure to JAK-inhibitors during pregnancy and risk of adverse pregnancy outcome

Case 3:

Safety and efficacy of JAK inhibitors in patients with primary sclerosing cholangitis associated with inflammatory bowel disease.

For more information on the format and content criteria, as well as the selection process, please find the full call below.

ECCO CONFER Call for Similar Cases

Deadline for similar cases: March 15, 2024.

Please  respond to this call by sending your case to This email address is being protected from spambots. You need JavaScript enabled to view it..

Should you have any further questions, do not hesistate to call or contact the ECCO Office via email.

Please find below further information on the cases.

Round 10 - Cases


About

Unique and extraordinary clinical cases are generally reported in the form of a single case report or a small series, making it hard to derive clinical conclusions on modes of presentation, diagnosis, and treatment.

5 main themes of interest:

  • Unusual clinical situations facing diagnostic or therapeutic gaps of knowledge
  • Rare infections
  • Uncommon drug beneficial effects or side-effects
  • Rare IBD manifestations
  • Infrequent disease associations (neoplastic, infectious etc.)

What is ECCO CONFER Cases?

The ECCO CONFER Cases are based on an initiative introduced by the European Crohn’s and Colitis Organisation (ECCO) to support individual investigators in developing rare case series by collecting enough similar cases among the IBD Community. By joining forces of the many members and supporters of ECCO, a joint report of all similar such cases can result in a large case series that will advance knowledge on these uncommon patients.

How it operates

A call will be made once a year to all ECCO Members to propose CONFER case projects. Two to four cases will be selected by the CONFER Steering Committee.

The proposing investigator will act as PI and develop his/her case into a CONFER case project with the support of a member of the CONFER Steering Committee.

A call will then be made to all ECCO Members and affiliates who have encountered a similar case to contribute their case to the CONFER case series. 

All received cases will be joined into a case series by a PI and prepared for publication.

ECCO’s support:

ECCO’s support includes dissemination of a call for similar cases, as well as assessment of the feasibility of the cases by the ECCO CONFER Steering Committee. It does not include any financial support nor any input in the collection of the data, the analysis or the publication of the data collected. 

Steering committee

ECCO CONFER is managed by a four-member steering committee. The current steering committee is composed of:

David Drobne (Slovenia)
Triana Lobaton (Belgium) 
Mette Julsgaard (Denmark)
Daniela Pugliese (Italy)

      


Previous Rounds of CONFER Cases

CONFER Cases Round 9
Case 1
Case Title Treatment of chronic pouchitis with tofacitinib
Principal Investigator Giulia Testa
Case Manager Triana Lobaton
Case Description The aim of this ECCO CONFER project is to perform an observational retrospective study in order to evaluate in order to evaluate the efficacy of tofacitinib as a treatment for chronic antibiotic refractory pouchitis (CARP). The objective is to recruit as most as possible cases in Europe to assess the effectiveness and safety of this drug usually used in ulcerative colitis. The primary outcome is the corticosteroid-free clinical response at 3 months (defined as modified Pouchitis activity Index score-mPDAI-reduction, considering the clinical symptoms). Secondary outcomes are: corticosteroid-free clinical response at 1 year (clinical symptoms part of mPDAI reduction by ≥ 2 points), corticosteroid-free clinical remission (clinical symptoms part of mPDAI score=0) at 3 months and at 1 year, endoscopic response (PDAI Endoscopic Inflammation subscore <3) at 1 year, endoscopic remission (defined as endoscopic PDAI ≤ 1) at 1 year, collection of side effects and trend of protein C-reactive and faecal calprotectin.
Main Clinical Questions CARP is an important clinical condition affecting a relevant percentage of patients with UC, who already underwent total colectomy. Infliximab and recently vedolizumab are the two more studied biologics in this setting, with few data about ustekinumab. Based on these facts, it’s clear that management of CARP is an important unmet need. To assess if these patients benefit from treatment with TOFA could change our clinical management of CARP.
Literature on the Topic Currently, data about the outcome of treatment of CARP with TOFA are very limited, with a total number of 4 reported patients. In particular, there is a case report in the literature in which a 20-year-old woman affected by refractory chronic pouchitis was successfully treated with tofacitinib, leading to clinical and endoscoping improvement (Okano S, Yoshimura N, Sako M, Takazoe M. A case of refractory chronic pouchitis successfully treated with tofacitinib. Clin J Gastroenterol. 2020 Aug;13(4):560-563. doi: 10.1007/s12328-020-01108-5. Epub 2020 Mar 4. PMID: 3213065.9). In addition to that, there are case series in 3 patients with chronic pouchitis were treated with TOFA. Clinical response was observed in 1 patient and endoscopic response was assessed in 1 of 2 patients (Dalal RS, Bains K, Marcus J, McClure EL, Allegretti JR. Tofacitinib for the Treatment of Pouch-Related Disorders: A Case Series. Inflamm Bowel Dis. 2022 Jul 6:izac147. doi: 10.1093/ibd/izac147. Epub ahead of print. PMID: 35792486).

Case 2
Case Title Safety and efficacy of ustekinumab and vedolizumab after liver transplant for PSC-IBD patients
Principal Investigator Tom Holvoet 
Case Manager Triana Lobaton
Case Description Around 5% of IBD patients develop primary sclerosing cholangitis (PSC), a chronic inflammatory disorder of the liver which can result in the need for liver transplantation. The management of intestinal disease activity after a liver transplantation can be challenging due to concomitant antirejection therapy and increased risk of infections. The newer biologicals (ustekinumab and vedolizumab) have shown good safety profile and therefore they can be an interesting alternative in this group of patients. However, data on the efficacy and safety in this setting is limited.
Main Clinical Questions The aim is to assess the safety and effectiveness of vedolizumab and ustekinumab in IBD patients with concurrent PSC who underwent a liver transplantation. Since this patients undergo a yearly surveillance colonoscopy and regular blood analysis, we expect to collect sufficient and qualitative data.
Literature on the Topic On vedolizumab1-10 - On ustekinumab11,12
1. Meszaros M, Pageaux G-P, Altwegg R. Management of ulcerative colitis using vedolizumab after liver transplantation for primary sclerosing cholangitis. Journal of Crohn's & colitis 2015;10:236-.
2. Wright AP, Fontana RJ, Stidham RW. Vedolizumab is safe and effective in moderate‐to‐severe inflammatory bowel disease following liver transplantation. Liver Transplantation 2017;23:968-71.
3. Hartery K, O'Reilly S, Houlihan D, et al. Vedolizumab for the management of inflammatory bowel disease in patients after liver transplantation for primary sclerosing cholangitis. Aliment Pharmacol Ther 2017;45:376-8.
4. Lim TY, Pavlidis P, Gulati S, et al. Vedolizumab in inflammatory bowel disease associated with autoimmune liver disease pre-and postliver transplantation: A case series. Inflamm Bowel Dis 2016;22:E39-E40.
5. Olmedo-Martin RV, Amo-Trillo V, Gonzalez-Grande R, Jimenez-Perez M. Efficacy and safety of vedolizumab as a treatment option for moderate to severe refractory ulcerative colitis in two patients after liver transplant due to primary sclerosing cholangitis. Revista Espanola de Enfermadades Digestivas (REED) 2017;109:659- 63.
6. Spadaccini M, Aghemo A, Caprioli F, et al. Safety of vedolizumab in liver transplant recipients: A systematic review. United European gastroenterology journal 2019;7:875-80.
7. Drastich P, Brezina J, Bajer L, Benes M, Spicak J. Vedolizumab for uc after infliximab failure in psc patients after oltx: Two case reports. In: JOURNAL OF CROHNS & COLITIS, 2018.
8. Daffra P, Etchevers M, Sobrero M, et al. P140 use of vedolizumab in a transplanted liver patient: A case report of the first experience in a liver transplant referral center in argentina. Journal of Crohn's and Colitis 2017;11.
9. Mumtaz S, Goh J, Hirschfield GM, Ferguson J, Cooper SC. Evolving strategies to reduce colectomy rates in primary sclerosing cholangitis-inflammatory bowel disease: Clinical remission of corticosteroid refractory colitis post-liver transplant with vedolizumab. Frontline gastroenterology 2016;7:271-4.
10. Al Draiweesh S, Ma C, Alkhattabi M, et al. A126 safety of combination biologic and immunosuppressive therapy post-orthotopic liver transplantation in patients with inflammatory bowel disease: A systematic review. Journal of the Canadian Association of Gastroenterology 2019;2:253-4.
11. Shibuya T, Nomura O, Nagahara A. Safety and efficacy of ustekinumab for ulcerative colitis in a liver transplant patient. Inflamm Bowel Dis 2021.
12. Martínez-Montiel M, Piedracoba-Cadahia P, Gómez-Gómez C, Gonzalo J. Ustekinumab is effective and safe in the treatment of crohn’s disease refractory to anti-tnfα in an orthotopic liver transplant patient. Journal of Crohn's and Colitis 2015;9:816-7.
Case 3
Case Title Biologicals and/or small molecules in patients with microscopic colitis
Principal Investigator Bram Verstockt
Case Manager Mette Julsgaard
Case Description Corticosteroid refractory microscopic colitis is a rare condition with an unknown prevalence, and with
very limited efficacy data in patients treated with biologicals and small molecules. Some case series report
some efficacy, but due to the lack of any trial data evidence is very limited. Hence, an ECCO confer series
reporting some real-world evidence would support the use of these drugs is refractory cases. In case the
ECCO confer steering committee considers the current proposal to extensive, we could also consider to
focus only on the efficacy of small molecules.
Main Clinical Questions Efficacy (and safety) of biologicals and/or small molecules in patients with microscopic colitis
Literature on the Topic

Mainly focussing on cases and prevalence, not on outcome:

Esteve et al. JCC 2011. Case series with efficacy on infliximab (n=5)
- Aram et al. CGH 2010. Case series with efficacy on anti-TNF therapy (n=2)
- Munch et al. Scan J Gastroenterol 2012. Case series with efficacy on adalimumab (n=3)
- Anderson et al. BMJ Case Rep 2016. Case report with efficacy on adalimumab (n=1)
- Daferera et al. UEGJ 2019. Case series with efficacy on infliximab (n=18)
- Rivière et al. JCC 2019. Case series with efficacy on vedolizumab (n=11)
- Jennings et al. IBD 2019. Case series with efficacy on vedolizumab (n=3)

Deadline for reporting similar cases: CLOSED

Please report your cases by sending an email to the ECCO Office (This email address is being protected from spambots. You need JavaScript enabled to view it.).


If the call proves the projects feasible by minimal number of similar cases available, transformation of the cases by the Principal Investigator and the CONFER Case Manager into standardised data-entry format (CRF) will next be performed. The CRFs will then be shared with the potential contributors who reported a similar case.