TY - JOUR
T1 - A rare case of Crohn’s ileitis in a patient with constitutional mismatch repair deficiency
AU - Kaimakliotis, Pavlos
AU - Giardiello, Francis
AU - Eze, Ogechukwu
AU - Truta, Brindusa
N1 - Publisher Copyright:
© 2017 Hellenic Society of Gastroenterology.
PY - 2017
Y1 - 2017
N2 - Constitutional mismatch repair deficiency (CMMRD), a variant of Lynch syndrome, is a rare disease characterized by café-au-lait spots, oligopolyposis, glioblastoma and lymphoma. A 24-year-old male, under surveillance for CMMRD, developed Crohn’s ileitis after total colectomy with end ileostomy for colorectal cancer and failed to respond to oral corticosteroids. The patient underwent induction and maintenance of remission with vedolizumab infusions. We report the first patient with CMMRD developing Crohn’s disease. The choice of immunosuppressive therapy in these patients is challenging and needs to be made according to their risk for malignancy.
AB - Constitutional mismatch repair deficiency (CMMRD), a variant of Lynch syndrome, is a rare disease characterized by café-au-lait spots, oligopolyposis, glioblastoma and lymphoma. A 24-year-old male, under surveillance for CMMRD, developed Crohn’s ileitis after total colectomy with end ileostomy for colorectal cancer and failed to respond to oral corticosteroids. The patient underwent induction and maintenance of remission with vedolizumab infusions. We report the first patient with CMMRD developing Crohn’s disease. The choice of immunosuppressive therapy in these patients is challenging and needs to be made according to their risk for malignancy.
KW - Constitutional mismatch repair deficiency
KW - Crohn’s disease
KW - Familial cancer
KW - Vedolizumab
UR - http://www.scopus.com/inward/record.url?scp=85018361683&partnerID=8YFLogxK
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U2 - 10.20524/aog.2017.0129
DO - 10.20524/aog.2017.0129
M3 - Article
C2 - 28469372
AN - SCOPUS:85018361683
SN - 1108-7471
VL - 30
SP - 370
EP - 372
JO - Annals of Gastroenterology
JF - Annals of Gastroenterology
IS - 3
ER -