TY - JOUR
T1 - Clinical phenotype and mortality in patients with idiopathic small bowel villous atrophy
T2 - A dual-centre international study
AU - Schiepatti, Annalisa
AU - Sanders, David S.
AU - Aziz, Imran
AU - De Silvestri, Annalisa
AU - Goodwin, John
AU - Key, Tim
AU - Quaye, Lydia
AU - Giuffrida, Paolo
AU - Vanoli, Alessandro
AU - Paulli, Marco
AU - Cross, Simon S.
AU - Vergani, Patricia
AU - Betti, Elena
AU - Maiorano, Gregorio
AU - Ellis, Richard
AU - Snowden, John A.
AU - Di Sabatino, Antonio
AU - Corazza, Gino R.
AU - Biagi, Federico
PY - 2020
Y1 - 2020
N2 - Objective Causes of small-bowel villous atrophy (VA) include coeliac disease (CD), its complications and other rare non-coeliac enteropathies. However, forms of VA of unknown aetiology may also exist. We defined them as idiopathic VA (IVA). To retrospectively classify the largest cohort of IVA patients and compare their natural history with CD. Methods Notes of 76 IVA patients attending two tertiary centres between January 2000 and March 2019 were retrospectively reviewed. CD, its complications and all the known causes of VA were excluded in all of them. Persistence of VA during follow-up and lymphoproliferative features were used to retrospectively classify IVA, as follows. Group 1: IVA with spontaneous histological recovery (50 patients). Group 2: persistent IVA without lymphoproliferative features (14 patients). Group 3: persistent IVA with lymphoproliferative features (12 patients). Survival was compared between IVA groups and 1114 coeliac patients. HLA was compared between IVA patients, coeliac patients and appropriate controls. Results Five-year survival was 96% in IVA group 1, 100% in IVA group 2, 27% in IVA group 3 and 97% in CD. On a multivariate analysis hypoalbuminemia (P = 0.002) and age at diagnosis (P = 0.04) predicted mortality in IVA. Group 2 showed association with HLA DQB1∗0301 and DQB1∗06. Conclusion IVA consists of three groups of enteropathies with distinct clinical phenotypes and prognoses. Mortality in IVA is higher than in CD and mainly due to lymphoproliferative conditions necessitating more aggressive therapies.
AB - Objective Causes of small-bowel villous atrophy (VA) include coeliac disease (CD), its complications and other rare non-coeliac enteropathies. However, forms of VA of unknown aetiology may also exist. We defined them as idiopathic VA (IVA). To retrospectively classify the largest cohort of IVA patients and compare their natural history with CD. Methods Notes of 76 IVA patients attending two tertiary centres between January 2000 and March 2019 were retrospectively reviewed. CD, its complications and all the known causes of VA were excluded in all of them. Persistence of VA during follow-up and lymphoproliferative features were used to retrospectively classify IVA, as follows. Group 1: IVA with spontaneous histological recovery (50 patients). Group 2: persistent IVA without lymphoproliferative features (14 patients). Group 3: persistent IVA with lymphoproliferative features (12 patients). Survival was compared between IVA groups and 1114 coeliac patients. HLA was compared between IVA patients, coeliac patients and appropriate controls. Results Five-year survival was 96% in IVA group 1, 100% in IVA group 2, 27% in IVA group 3 and 97% in CD. On a multivariate analysis hypoalbuminemia (P = 0.002) and age at diagnosis (P = 0.04) predicted mortality in IVA. Group 2 showed association with HLA DQB1∗0301 and DQB1∗06. Conclusion IVA consists of three groups of enteropathies with distinct clinical phenotypes and prognoses. Mortality in IVA is higher than in CD and mainly due to lymphoproliferative conditions necessitating more aggressive therapies.
KW - human leukocyte antigen
KW - mortality
KW - non-coeliac enteropathies
KW - villous atrophy
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U2 - 10.1097/MEG.0000000000001726
DO - 10.1097/MEG.0000000000001726
M3 - Article
C2 - 32282540
AN - SCOPUS:85086165890
SP - 938
EP - 949
JO - European Journal of Gastroenterology and Hepatology
JF - European Journal of Gastroenterology and Hepatology
SN - 0954-691X
ER -