Annalisa Schiepatti1,2, David S Sanders2, Imran Aziz2, Annalisa De Silvestri3, John Goodwin4, Tim Key4, Lydia Quaye5, Paolo Giuffrida6, Alessandro Vanoli7, Marco Paulli7, Simon S Cross8, Patricia Vergani8, Elena Betti6, Gregorio Maiorano6, Richard Ellis9, John A Snowden10, Antonio Di Sabatino6, Gino R Corazza6, Federico Biagi1. 1. Istituti Clinici Scientifici Maugeri, IRCCS, Gastroenterology Unit of Pavia Institute, University of Pavia, Pavia, Italy. 2. Academic Unit of Gastroenterology, Royal Hallamshire Hospital, Sheffield, UK. 3. Clinical Epidemiology, IRCCS San Matteo Hospital Foundation, Pavia, Italy. 4. Histocompatibility and Immunogenetics Laboratory, NHS Blood and Transplant, Sheffield. 5. Histocompatibility and Immunogenetics, NHS Blood and Transplant, Colindale, London, UK. 6. First Department of Internal Medicine, IRCCS Policlinico San Matteo, University of Pavia. 7. Anatomic Pathology Unit, Department of Molecular Medicine and IRCCS San Matteo Hospital, Pavia, Italy. 8. Department of Histopathology, Royal Hallamshire Hospital, Sheffield. 9. Department of Gastroenterology, Queen Alexandra Hospital, Portsmouth. 10. Department of Haematology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.
Abstract
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.
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.
Authors: Annalisa Schiepatti; Anupam Rej; Stiliano Maimaris; Simon S Cross; Petra Porta; Imran Aziz; Tim Key; John Goodwin; Amelie Therrien; Shakira Yoosuf; Daniel A Leffler; Jocelyn A Silvester; Catherine Klersy; Federico Biagi; David S Sanders Journal: Aliment Pharmacol Ther Date: 2021-09-08 Impact factor: 8.171
Authors: Annalisa Schiepatti; David S Sanders; Paola Baiardi; Giacomo Caio; Carolina Ciacci; Katri Kaukinen; Benjamin Lebwohl; Daniel Leffler; Georgia Malamut; Joseph A Murray; Kamran Rostami; Alberto Rubio-Tapia; Umberto Volta; Federico Biagi Journal: Gut Date: 2022-06-08 Impact factor: 31.793