P253 De novo Inflammatory Bowel Disease following paediatric liver transplantation: A case series of three patients and world literature review
K. Nikaki*1, D.C. Wilson2, P. McKiernan3, C.H. Spray4
1Birmingham Children's Hospital NHS Foundation Trust, Paediatric Liver Unit, Birmingham, United Kingdom, 2Royal Hospital for Sick Children, Paediatric Gastroenterology and Nutrition Department , Edinburgh, United Kingdom, 3Birmingham Children's Hospital NHS Foundation Trust, Paediatric Liver Unit , Birmingham, United Kingdom, 4Bristol Royal Hospital for Children, Paediatric Gastroenterology Department, Bristol, United Kingdom
Risk Factors for De Novo IBD and Immunosuppressive Rx before and after De Novo IBD Diagnosis
|Case 1: Crohn’s disease||Case 2: Indeterminate Colitis||Case 3: Crohn’s disease|
|CMV mismatch (donor / recipient)||-ve/-ve||+ve / -ve||-ve/-ve|
|CMV infection post LTx||No||No||No|
|Acute / Chronic Rejection||No / Yes||No / No||No / No|
|PMHx of Autoimmunity||No||No||No|
|FHx of Autoimmunity||No||No||No|
|Immunosuppression at presentation with de novo IBD||Tacrolimus, Prednisolone, MMF||Cyclosporine||Cyclosporine|
|Immunosuppression after diagnosis with de novo IBD||Tacrolimus, Prednisolone, AZA||MMF, Prednisolone||MMF, Prednisolone|
|Other IBD Tx following diagnosis||Infliximab||Mesalazine||Mesalazine|
Inflammatory Bowel Disease (IBD) is a T-cell driven inflammatory process due to inappropriate and enduring activation of the enteric immune system, generally treated with immunosuppressant therapy. Following solid organ transplantation (SOT), recurrent and de novo IBD have been described despite immunosuppressive therapy. The majority of cases in adult patients occurred post liver transplantation (LT) (136/175) with 96/136 cases reported having been originally transplanted for sclerosing cholangitis (SC) or autoimmune hepatitis (AIH). In paediatrics, 14 cases have been described post liver, heart and renal transplant. 9 cases have been described post LT (3/9 cases transplanted for SC/AIH). Various risk factors have been implicated in the development of post-transplant IBD. Herein, we describe 3 cases of de novo IBD post LT for causes other than SC/AIH.
Case 1 was the index case and the other 2 patients were identified through an electronic search of the Birmingham Liver Unit Paediatric Transplant database that holds the data of 782 patients who have undergone a liver transplant between 1983 and 2014. Medline and Embase were searched for "de novo inflammatory bowel disease" and "transplantation". The search was extending by scanning reference lists of related articles and free text web search. A total of 46 articles were included in the systematic review.
3 patients (2 females) were identified with de novo IBD following LT. 2 patients were originally transplanted for a1 antitrypsin deficiency and 1 for extra-hepatic biliary atresia. Risk factors for the development of post LT IBD are shown in table 1.
Mean age at the time of de novo IBD diagnosis was 7.3 years (range 3-11 years) with a mean of 4.6 years post LT. Diarrhoea was the presenting symptom in 2 patients and intermittent rectal bleeding in 1. All patients were investigated for possible IBD according to Porto criteria. The patients underwent on average 2.6 upper and/or lower GI endoscopies prior to diagnosis, while the time of presentation to diagnosis varied from 3 months to 1 year. Crohn’s Disease was diagnosed in 2 patients and Indeterminate Colitis in 1. Infliximab was used in 1 patient while the other 2 were treated with 5-aminosalicylic acids. All patients are in clinical remission.
De novo IBD does occur following liver transplantation in children but is rare. De novo IBD should be considered in the differential diagnosis of chronic diarrhoea post-transplant.