(Copenhagen, 25 June 2022) Patients with a paediatric onset immune-mediated inflammatory disease (pIMID) have a significantly higher risk of premature death, according to new research1 being presented today at the 54th Annual Meeting of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN).
Whilst higher mortality was found in patients across all included paediatric onset immune-mediated inflammatory conditions compared to controls, paediatric autoimmune liver disease (pAILD) and paediatric vasculitis patients had the highest risk of mortality with a fourteen times (aHR* 14.3) and fifteen times (aHR 15.8) greater chance of death respectively.
For pAILD patients specifically, the study reveals for the very first time that the high death rate was driven by the risk of cancer, which was thirty times greater in pAILD patients. Coupled with the 6-times increased risk of death from cancer in paediatric onset inflammatory bowel disease (pIBD) patients, the researchers believe the findings show a definitive need to establish early cancer screening in pAILD and pIBD patients to prevent unnecessary premature deaths.
The study also revealed a significantly higher suicide risk (almost two-and-a-half times greater [aHR 2.4]) amongst pIMID patients compared to controls. Primarily driven by pIBD and juvenile idiopathic arthritis (JIA) patients, the median age of suicide was just 25 years.
These findings show a possible impact on the mental health of patients, spotlighting the true burden of these conditions. As the majority of suicides occurred in patients after transfer into adult care, an increased focus is warranted on systematic transitioning programmes in paediatric departments. This focus must be continued into the period after the transfer of the patient to an adult department, due to this critical life period.
The survey also showed that being diagnosed with more than one IMID appears to be a risk factor, with significantly higher mortality risk found in these patients (aHR 9.2). This is important as previous studies have found that patients diagnosed with one IMID are at an increased risk of subsequently being diagnosed with an additional IMID.2
The Danish population-based study recorded data from 12,036 pIMID patients between 1980 – 2018, consisting of 5,671 (47%) pIBD, 396 (3%) pAILD, 6,018 (50%) JIA, and 300 (2%) individuals with paediatric onset vasculitis. Of these, 342 (3%) individuals were diagnosed with more than one pIMID.
Commenting on the findings, lead author, Dr Mikkel Malham from the Department of Paediatrics and Adolescent Medicine at the Copenhagen University Hospital Hvidovre in Copenhagen, Denmark, stated: “This is the first study to report an increased mortality in pIMID. While for pIBD this risk is quite well known, for the rest of the included pIMIDs the presented risk estimates should raise considerable concern.”
“The increased risk of dying from several different causes should warrant a multidisciplinary approach which includes caring for a child’s mental health. It is of utmost importance that this multidisciplinary approach is continued into early adulthood, as this is when suicide typically occurs.”
“Additionally, cancer screening in IMID patients diagnosed in childhood, particularly with IBD and AILD, should probably be initiated early to prevent premature death”, adds Dr Malham.
Referencing the higher mortality rate, particularly amongst pAILD patients, and the prominent role of suicide as a cause, Chair of the ESPGHAN Hepatology Committee, Professor Giuseppe Indolfi elaborates on the broader implications: “The clinical and therapeutic management of children and adolescents with autoimmune liver and gastrointestinal diseases remains a significant challenge for paediatric hepatologists and gastroenterologists. This study reinforces that every effort should be made to further improve our knowledge and ultimately the quality of care for children with immune-mediated inflammatory diseases.”
*adjusted Hazard Ratio
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Notes to Editors
In this study cases were matched with up to 10 controls from the general population based on age, sex, and place of residence. Cases and controls were linked with the Cause of Death register identifying all patients who subsequently died, including cause of death. Risk of premature death was calculated by Cox Proportional Hazard Model and presented by adjusted Hazard Ratios (aHR). Cases and controls were followed through the registries for a median of 11.2 years and 10.5 years, respectively.
For further information, to speak to Dr Mikkel Malham or an ESPGHAN expert, please contact Sean Deans at [email protected] or call +44 (0) 208 154 6396.
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About the Expert
Dr Mikkel Malham is from the Department of Paediatrics and Adolescent Medicine at the Copenhagen University Hospital – Hvidovre, Denmark
The European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) is a multi-professional organisation whose aim is to promote the health of children with special attention to the gastrointestinal tract, liver and nutritional status, through knowledge creation, the dissemination of science based information, the promotion of best practice in the delivery of care and the provision of high quality education for paediatric gastroenterology, hepatology and nutrition professionals in Europe and beyond. Find out more by visiting www.espghan.org
About the 54th Annual Meeting of ESPGHAN
The 54th Annual Meeting of ESPGHAN is taking place from 23-25 June 2022, in Copenhagen, Denmark. Every year the ESPGHAN Annual Meeting attracts over 4,600 experts and key opinion leaders in the field of Paediatric Gastroenterology, Hepatology and Nutrition from 100 countries across Europe and all five continents, turning it into the largest conference of its kind worldwide.
For more information about the ESPGHAN Annual Meeting, including to view the programme, please visit: https://www.espghancongress.org/
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- Malham M, et al. (2022). Mortality among patients with paediatric-onset immune-mediated inflammatory disease – a nationwide study from Denmark. Presented at the 54th Annual Meeting of ESPGHAN in Copenhagen, 22–25 June 2022.
- Aletaha D, et al. Risk of Developing Additional Immune-Mediated Manifestations: A Retrospective Matched Cohort Study. Adv Ther. 2019;36:1672–83.