Abstract
Enteropathy may be the first presentation of immunodeficiency or it may occur during the course of the disease and in association with malabsorption in patients affected by primary antibody diseases. For these patients, immunoglobulin G (IgG) replacement therapy prevents infectious and non-infectious complications. Nonetheless some patients cannot achieve optimal IgG trough levels, even when treated with high Ig doses in absence of protein-losing syndromes. We investigated seven patients affected by common variable immunodeficiencies (CVIDs) and treated with high Ig doses (600–800 mg/kg/month) showing low IgG trough level. Patients underwent abdominal scintigraphy with human polyclonal immunoglobulin G labeled with 99mTc and with white blood cells labeled by 111 Indium-oxinate to investigate asymptomatic bowel inflammation. A concentration of labeled leukocytes in abdominal segments greater than that observed with human polyclonal immunoglobulin G was evident only in one patient. In five patients a slight concentration of both radiopharmaceuticals was reported, due to mild intestinal inflammatory response. These data might be related to mild increase of capillary permeability in the absence of inflammation leukocyte mediated. This study discloses a new cause of IgG-accelerated catabolism due to inflammatory bowel conditions without diarrhea in CVID patients.
Highlights
Common variable immunodeficiencies (CVIDs) are the most common symptomatic primary immunodeficiencies in adults
We studied seven patients (6 males and 1 female) affected by CVIDs diagnosed according to current European Society for Immunodeficiencies (ESID) criteria [1]
In 7/24 segments we observed the same positive uptake of white blood cells (WBCs) and 99mTc-HIG; in 2/24 segments the leukocytes uptake exceeded the uptake of immunoglobulins
Summary
Common variable immunodeficiencies (CVIDs) are the most common symptomatic primary immunodeficiencies in adults. The disease is characterized by low levels of serum immunoglobulin G (IgG), IgA and/or IgM, impaired specific antibody production in response to vaccination, and higher increased risk of bacterial infections associated to non-infectious complications. CVID patients present respiratory tract infections, autoimmunity, cancers, and gastrointestinal (GI) diseases. GI diseases include chronic atrophic gastritis, pernicious anemia, nodular lymphoid hyperplasia, chronic diarrhea, inflammatory bowel disease, celiac-like disease, lymphomas, gastric adenocarcinoma, and non-specific malabsorption [3]. Gastrointestinal inflammatory diseases can be present even in pauci-symptomatic patients [4]. Enteropathy may be the first clinical presentation of immunodeficiency [5] or it may occur during the course of the disease affecting large and/or small bowels and it might be associated with malabsorption [6]
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