{
  "abstract": "Introduction Hypogammaglobulinemia represents a heterogeneous group of immunodeficiency disorders caused by low serum antibodies, resulting in recurrent infections and increased susceptibility to autoimmune and inflammatory complications. Patients with significant infectious complications are treated with immunoglobulin infusions. Many patients with immunodeficiency have concurrent gastrointestinal (GI) symptoms. However, the nature of these symptoms and GI manifestations in these patients remains unclear. We aimed to report on GI manifestations in patients with hypogammaglobulinemia under the care of Sheffield Teaching Hospitals.Methods Electronic patient records of patients with hypogammaglobulinaemia currently receiving IV immunoglobuin replacement under the care of Sheffield Teaching Hospitals were retrospectively reviewed. Data was collected regarding GI symptoms, manifestations and eventual GI diagnoses.Results Fourty-eight patients on immunoglobulin infusions were included (median age 47 years, 50% female), of which 75% (n=36) of patients had common variable immunodeficiency (CVID), 23% (n=11) had other primary immunodeficiencies within the spectrum of hypogammaglobulinaemia and 2% (n=1) had secondary immunodeficiency. In total, 65% (n=31) of patients had GI symptoms. The most common GI symptom was diarrhoea, occurring in 46% (n=22) of patients, followed by weight loss in 13% (n=6), bloating (n=3) and vomiting (n=3). 35% (n=17) of patients were asymptomatic. In those with symptoms, the most frequent cause was infection, identified in 45% (14/31), including Giardia lamblia (n=4), Campylobacter jejuni (n=4) and Helicobacter pylori (n=2). Overall, non-infectious enteropathies were found in 6% (n=3) of patients: CVID-enteropathy (n=1), ulcerative colitis (n=1) and HIV-related enteropathy (n=1). Irritable bowel syndrome was diagnosed in 12.5% (n=6) and bile acid malabsorption in 6% (n=3) of patients. Liver abnormalities were identified in 15% patients (n=7), of which three had cryptogenic cirrhosis, later presumed to be related to immunodeficiency.Conclusion Gastrointestinal symptoms are prevalent in nearly two-thirds of patients with immunodeficiency on treatment, with almost half of them having diarrhoea. Infections remain the most frequent GI manifestation, presenting in a quarter of patients. Non-infectious enteropathies were present in only 6% of our cohort, whereas existing literature suggests a higher prevalence of around 10%, suggesting that these conditions may be under-recognised. Prevalence of bile acid diarrhoea was higher than in the general population, suggesting a potential association with immunodeficiency. Prospective studies are needed to determine the true spectrum of GI manifestations in hypogammaglobulinaemia.",
  "authors": [
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom"
      ],
      "name": "Kimberley Butler"
    },
    {
      "affiliations": [
        "Department of Immunology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom"
      ],
      "name": "Sarah Denness"
    },
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom"
      ],
      "name": "Anupam Rej"
    },
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom"
      ],
      "name": "Foong Way David Tai"
    },
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom"
      ],
      "name": "Hugo Penny"
    }
  ],
  "title": "P382 GI manifestations of hypogammaglobulinaemia: a retrospective study",
  "uid": "b9b03a50-5297-5fc5-a47a-af451afcfc26"
}