BACKGROUND: Common variable immunodeficiency (CVID) is characterized by hypogammaglobulinemia, defective antibody production, and recurrent upper and lower airway tract infections. OBJECTIVES: To reveal the clinical heterogeneity of this condition, analyze the high frequency of respiratory and gastrointestinal complications despite satisfactory trough immunoglobulin (Ig) G levels, and determine the main difficulties in management and treatment. METHODS: We performed a retrospective analysis of 23 patients (13 male and 10 female) diagnosed with CVID between 2001 and 2008. RESULTS: The median diagnostic delay for females and males was 15 years (range, 1-32 years) and 8 years (range, 1-31 years), respectively. Restrictive, obstructive, and combined pulmonary function defects were determined in 23%, 27%, and 14% of patients, respectively. The most frequent findings on the thoracic computed tomography scan were bronchiectasis, mediastinal lymphadenopathy, fibrosis, ground-glass patterns, mosaic oligemia, peribronchial cuffing, and parenchymal nodules. Giardiasis and duodenal lymphoid hyperplasia were detected in 52% and 42% of the patients, respectively, and Helicobacter pylori in 42%. Vitamin A levels were normal, although beta-carotene and/or vitamin E levels were decreased in patients presenting malabsorption-related symptoms. Malignancy was documented in 3 patients and decreased bone mineral density in 9 patients (3 had osteoporosis and 3 had osteomalacia). CONCLUSION: CVID is a multisystemic disease that should be managed by a multidisciplinary team. Intravenous immunoglobulin therapy and antibiotics do not seem to have a suppressive effect on granulomatous or inflammatory manifestations. More comprehensive studies based not only on peripheral blood but also on immunohistological analysis are necessary to shed light on the pathogenesis of these life-threatening complications.
BACKGROUND: Common variable immunodeficiency (CVID) is characterized by hypogammaglobulinemia, defective antibody production, and recurrent upper and lower airway tract infections. OBJECTIVES: To reveal the clinical heterogeneity of this condition, analyze the high frequency of respiratory and gastrointestinal complications despite satisfactory trough immunoglobulin (Ig) G levels, and determine the main difficulties in management and treatment. METHODS: We performed a retrospective analysis of 23 patients (13 male and 10 female) diagnosed with CVID between 2001 and 2008. RESULTS: The median diagnostic delay for females and males was 15 years (range, 1-32 years) and 8 years (range, 1-31 years), respectively. Restrictive, obstructive, and combined pulmonary function defects were determined in 23%, 27%, and 14% of patients, respectively. The most frequent findings on the thoracic computed tomography scan were bronchiectasis, mediastinal lymphadenopathy, fibrosis, ground-glass patterns, mosaic oligemia, peribronchial cuffing, and parenchymal nodules. Giardiasis and duodenal lymphoid hyperplasia were detected in 52% and 42% of the patients, respectively, and Helicobacter pylori in 42%. Vitamin A levels were normal, although beta-carotene and/or vitamin E levels were decreased in patients presenting malabsorption-related symptoms. Malignancy was documented in 3 patients and decreased bone mineral density in 9 patients (3 had osteoporosis and 3 had osteomalacia). CONCLUSION:CVID is a multisystemic disease that should be managed by a multidisciplinary team. Intravenous immunoglobulin therapy and antibiotics do not seem to have a suppressive effect on granulomatous or inflammatory manifestations. More comprehensive studies based not only on peripheral blood but also on immunohistological analysis are necessary to shed light on the pathogenesis of these life-threatening complications.
Authors: Jennifer J Meerburg; Ieneke J C Hartmann; Sigune Goldacker; Ulrich Baumann; Annette Uhlmann; Eleni-Rosalina Andrinopoulou; Mariette P C Kemner V/D Corput; Klaus Warnatz; Harm A W M Tiddens Journal: Front Immunol Date: 2020-10-30 Impact factor: 7.561