Περίληψη:
Background: Risk benefit strategies in managing inflammatory bowel
diseases (IBD) are dependent upon understanding the risks of
uncontrolled inflammation vs those of treatments. Malignancy and
mortality in IBD have been associated with disease-related inflammation
and immune suppression, but data are limited due to their rare
occurrence.
Aim: To identify and describe the most common causes of mortality, types
of cancer and previous or current therapy among children and young
adults with paediatric-onset IBD.
Methods: Information on paediatric-onset IBD patients diagnosed with
malignancy or mortality was prospectively collected via a survey in 25
countries over a 42-month period. Patients were included if death or
malignancy occurred after IBD diagnosis but before the age of 26years.
Results: In total, 60 patients were identified including 43 malignancies
and 26 fatal cases (9 due to cancer). Main causes of fatality were
malignancies (n=9), IBD or IBD-therapy related nonmalignant causes
(n=10; including 5 infections), and suicides (n=3). Three cases, all
fatal, of hepatosplenic T-cell lymphoma were identified, all were
biologic-naive but thiopurine-exposed. No other haematological
malignancies were fatal. The 6 other fatal cancer cases included 3
colorectal adenocarcinomas and 3 cholangiocarcinomas (CCAs). Primary
sclerosing cholangitis (PSC) was present in 5 (56%) fatal cancers (1
colorectal carcinoma, 3 CCAs and 1 hepatosplenic T-cell lymphoma).
Conclusions: We report the largest number of paediatric-onset IBD
patients with cancer and/or fatal outcomes to date. Malignancies
followed by infections were the major causes of mortality. We identified
PSC as a significant risk factor for cancer-associated mortality.
Disease-related adenocarcinomas were a commoner cause of death than
lymphomas.
Συγγραφείς:
Joosse, M. E.
Aardoom, M. A.
Kemos, P.
Turner, D. and
Wilson, D. C.
Koletzko, S.
Martin-de-Carpi, J.
Fagerberg, U.
L.
Spray, C.
Tzivinikos, C.
Sladek, M.
Shaoul, R. and
Roma-Giannikou, E.
Bronsky, J.
Serban, D. E.
Ruemmele, F. M.
and Garnier-Lengline, H.
Veres, G.
Hojsak, I.
Kolho, K. L.
and Davies, I. H.
Aloi, M.
Lionetti, P.
Hussey, S. and
Veereman, G.
Braegger, C. P.
Trindade, E.
Wewer, A. V. and
Hauer, A. C.
de Vries, A. C. H.
Boneh, R. Sigall
Shabat, C.
Sarbagili
Levine, A.
de Ridder, L.
Paediat IBD Porto Grp
ESPGHAN
