The high mortality of patients with common variable immunodeficiency and small bowel villous atrophy

Pensieri, Maria Vittoria; Pulvirenti, Federica; Schiepatti, Annalisa; Maimaris, Stiliano; Lattanzio, Salvatore; Quinti, Isabella; Klersy, Catherine; Corazza, Gino Roberto; Biagi, Federico

doi:10.1080/00365521.2019.1568543

Cited by 19 publications

(13 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Age and hypoalbuminaemia at time of diagnosis of VA are important predictors of mortality in IVA and may be helpful to stratify patients warranting a more aggressive treatment and close follow-up. Our results on IVA add to some previous studies suggesting that overall mortality in non-coeliac enteropathies with VA is higher than in in CD [5,8,10,32].…”

Section: Discussionsupporting

confidence: 86%

Clinical phenotype and mortality in patients with idiopathic small bowel villous atrophy: a dual-centre international study

Schiepatti

Sanders

Aziz

et al. 2020

European Journal of Gastroenterology &Amp; Hepatology

Self Cite

View full text Add to dashboard Cite

show abstract

Section: Discussionsupporting

confidence: 86%

Clinical phenotype and mortality in patients with idiopathic small bowel villous atrophy: a dual-centre international study

Schiepatti

Sanders

Aziz

et al. 2020

European Journal of Gastroenterology &Amp; Hepatology

Self Cite

View full text Add to dashboard Cite

show abstract

“…Of great concern, is that CVID patients with chronic enteropathy and significant malabsorption have a known increase in mortality, as shown in several studies. [15,16] The pathology in CVID enteropathy is characteristic for an increase in intraepithelial CD8 + T cell infiltrates in the intestinal lamina propria, increased IL-12 and IFNγ secretion [17], and a paucity or absence of plasma cells [10,18]. We have also found increased IFNγ secreting innate lymphoid cells (ILC) in blood, respiratory and gastrointestinal mucosa of CVID patients with inflammatory conditions [19].…”

Section: Discussionmentioning

confidence: 78%

Vedolizumab therapy in common variable immune deficiency associated enteropathy: A case series

Sifers

Hirten

Mehandru

et al. 2020

Clinical Immunology

View full text Add to dashboard Cite

A number of gastrointestinal complications occur in common variable immunodeficiency (CVID). Infections are one cause, but various forms of severe non-infectious enteropathy also lead to substantial morbidity. The presence of T cell lymphocytic infiltrates in the mucosa have suggested that vedolizumab, a humanized monoclonal antibody which binds to alpha4 beta7 integrin and inhibits the migration of effector T-lymphocytes into gastrointestinal tissues, would be an effective treatment. A previous report of 3 CVID cases suggested benefit in 2 subjects. In this study 7 CVID patients with severe enteropathy were treated with vedolizumab. Four of the 7 completed vedolizumab induction therapy but 3 subjects had acute decompensation during induction and treatment was stopped. While one subject showed improvement, 6 of the 7 patients were withdrawn from therapy. While vedolizumab may be of use in some CVID subjects, it was not ultimately found helpful in most of these patients.

show abstract

“…In our series, this was the case for autoimmune enteropathy, tropical sprue and forms of idiopathic VA. 5,6,9,34,35 This is particularly relevant because of the higher mortality observed in non-coeliac SNVA compared to coeliac patients. 9,10,36 This study has some limitations. First, follow-up duodenal biopsies were not available in 40 patients with positive HLA-DQ2/-DQ8, no alternative causes for VA and satisfactory clinical response to a GFD.…”

Section: Seronegative Coeliac Disease and Coeliac Disease Associated Withmentioning

confidence: 94%

Clinical classification and long‐term outcomes of seronegative coeliac disease: a 20‐year multicentre follow‐up study

Schiepatti

Rej

Maimaris

et al. 2021

Aliment Pharmacol Ther

Self Cite

View full text Add to dashboard Cite

Background: Seronegative coeliac disease is poorly defined. Aims:To study clinical phenotypes and long-term outcomes of seronegative coeliac disease in a multicentre cohort over 20 years.Methods: Seronegative coeliac disease was diagnosed in HLA-DQ2/DQ8-positive patients with villous atrophy (VA), negative IgA endomysial (EmA), tissue transglutaminase (tTG) and deamidated-gliadin antibodies (DGP), clinical and histological response to a gluten-free diet (GFD), and no alternative causes for VA. In patients with IgA deficiency, coeliac disease was diagnosed through VA, positive IgG EmA/tTG/ DGP and clinical/histological response to a GFD (coeliac disease+IgAd). Patients with seropositive coeliac disease served as controls.Results: Of 227 patients previously diagnosed with seronegative coeliac disease, true seronegative coeliac disease was confirmed in 84, coeliac disease+IgAd in 48, and excluded in 55. Lack of follow-up duodenal biopsy precluded diagnosing seronegative coeliac disease in 40 patients. 2084 patients with seropositive coeliac disease served as controls. True seronegative coeliac disease had more severe symptoms at diagnosis and a higher risk of complications (HR 10.87,, P < 0.001) and mortality (HR 2.18, 95% CI 1.12-4.26, P < 0.01) than seropositive coeliac disease.There were no differences between true seronegative coeliac disease and coeliac disease+IgAd. On multivariate analysis, age at diagnosis, lack of clinical response to a GFD, true seronegative coeliac disease, coeliac disease+IgAd, and classical presentation predicted complications. Age at diagnosis, complications and absence of clinical response to a GFD predicted mortality. Conclusions:Seronegative coeliac disease has a more aggressive disease phenotype than seropositive coeliac disease. These data argue against over-reliance on serology for the diagnosis of coeliac disease and support a strict clinical and histologic followup in seronegative coeliac disease.

show abstract

The high mortality of patients with common variable immunodeficiency and small bowel villous atrophy

Cited by 19 publications

References 18 publications

Clinical phenotype and mortality in patients with idiopathic small bowel villous atrophy: a dual-centre international study

Clinical phenotype and mortality in patients with idiopathic small bowel villous atrophy: a dual-centre international study

Vedolizumab therapy in common variable immune deficiency associated enteropathy: A case series

Clinical classification and long‐term outcomes of seronegative coeliac disease: a 20‐year multicentre follow‐up study

Contact Info

Product

Resources

About