OBJECTIVES: Common variable immunodeficiency (CVID) is a primary humoral immunodeficiency characterised by reduced serum levels of immunoglobulins, recurrent infections, autoimmune phenomena and lymphoproliferative disorders. Gastrointestinal symptoms are very common in these patients and a coeliac-like villous atrophy was described in some of them. Since mortality in CVID is much higher than in the general population, our aim was to evaluate mortality rates and clinical predictors of survival in patients with both CVID and duodenal villous atrophy. PATIENTS AND METHODS: Sex, date of diagnosis of villous atrophy, HLA genomic typing, date of death/last follow-up, type of complication were retrospectively collected from medical files. Univariate analysis for each predictor was conducted and Kaplan-Meier curves were generated to evaluate survival. RESULTS: Twenty-three patients were enrolled (9 females, mean age at diagnosis of villous atrophy 38 ± 13 years) and 8 of them died after a median time of 96 months (25th-75th 60-120 months) corresponding to a mortality rate of 3.9 per 100 person-years (95% CI 1.9-7.7). Mortality was higher in men compared to women (60 vs. 11/1000 person-years), although not statistically significant. Causes of death included onco-haematological disorders and infections. CONCLUSIONS: Although based on a small cohort, our results confirm that patients with CVID and villous atrophy are burdened by a very high mortality mainly due to onco-immunological disorders and infections. Strict follow-up is required in these patients.
OBJECTIVES: Common variable immunodeficiency (CVID) is a primary humoral immunodeficiency characterised by reduced serum levels of immunoglobulins, recurrent infections, autoimmune phenomena and lymphoproliferative disorders. Gastrointestinal symptoms are very common in these patients and a coeliac-like villous atrophy was described in some of them. Since mortality in CVID is much higher than in the general population, our aim was to evaluate mortality rates and clinical predictors of survival in patients with both CVID and duodenal villous atrophy. PATIENTS AND METHODS: Sex, date of diagnosis of villous atrophy, HLA genomic typing, date of death/last follow-up, type of complication were retrospectively collected from medical files. Univariate analysis for each predictor was conducted and Kaplan-Meier curves were generated to evaluate survival. RESULTS: Twenty-three patients were enrolled (9 females, mean age at diagnosis of villous atrophy 38 ± 13 years) and 8 of them died after a median time of 96 months (25th-75th 60-120 months) corresponding to a mortality rate of 3.9 per 100 person-years (95% CI 1.9-7.7). Mortality was higher in men compared to women (60 vs. 11/1000 person-years), although not statistically significant. Causes of death included onco-haematological disorders and infections. CONCLUSIONS: Although based on a small cohort, our results confirm that patients with CVID and villous atrophy are burdened by a very high mortality mainly due to onco-immunological disorders and infections. Strict follow-up is required in these patients.
Entities:
Keywords:
Common variable immunodeficiency; coeliac disease; mortality; survival; villous atrophy
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: Ida Judyta Malesza; Michał Malesza; Iwona Krela-Kaźmierczak; Aleksandra Zielińska; Eliana B Souto; Agnieszka Dobrowolska; Piotr Eder Journal: Int J Mol Sci Date: 2020-07-23 Impact factor: 5.923
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