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Original Article
 

Clinical and Immunological Analysis of 23 Adult Patients With Common Variable Immunodeficiency
 

Ö Ardeniz,1 ÖK Bașoğlu,2 F Günșar,3 M Ünsel,1 S Bayraktaroğlu,4 N Mete1 O Gülbahar,1 A Sin1

1Ege University Medical Faculty, Department of Internal Medicine Division of Allergy and Clinical Immunology, Izmir, Turkey
2Ege University Medical Faculty, Department of Chest Diseases, Izmir, Turkey
3Ege University Medical Faculty, Department of Gastroenterology, Izmir, Turkey
4Ege University Medical Faculty, Department of Radiology, Izmir, Turkey

J Investig Allergol Clin Immunol 2010; Vol. 20(3): 222-236
 

 Abstract


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 ß-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.

Key words: CVID. Adults. Respiratory manifestations. Gastrointestinal manifestations. Granulomatous disease. IVIG therapy. Antibiotic prophylaxis.