PURPOSE: Common variable immune deficiency (CVID) is the primary immunodeficiency encountered most often in clinical practice. Patients have primary antibody deficiencies and present with recurrent sinopulmonary infections. Pulmonary disease is a common complication that may progress despite immunoglobulin replacement therapy. Our study focuses on defining the prevalence and spectrum of pulmonary disease in patients with CVID at our institution over the past 5 years (January 2006 to March 2011).
METHODS: A retrospective chart review was performed of all patients who were diagnosed with CVID at our institution from January 2006 to March 2011. Diagnosis of CVID was established according to the European Society for Immunodeficiencies (ESID) criteria (Conley, Clin Immunology, 1999). Pulmonary complications were defined as symptomatology and diagnoses found in the clinical data (medical records), abnormalities on lung function (pulmonary function tests), imaging (chest computed tomography), and pathology.
RESULTS: Fifty-one patients with CVID that met specific ESID criteria were identified. Of these, 31 were female and 20 were male. Age at diagnosis ranged from 10 to 83 years with a current median age of 42. 45 of the 51(88%) patients presented with recurrent respiratory complaints, five of which carried the diagnosis of asthma. Forty one patients (80%) had a high resolution chest CT performed. Findings included pulmonary nodules (n=13), nodular infiltrates/consolidation (n=13), fibrosis (n=9), lymphadenopathy (n=7), bronchiectasis (n=7), atelectasis (n=5), bronchial wall thickening (n=4), pleural effusion (n=3), emphysema (n=3), and mucous plugs (n=1). Pulmonary function tests were abnormal in 9/40(23%) patients (8 obstructive, 1 mixed pattern). Bronchoscopy was performed in ten patients with 7 transbronchial biopsies obtained. Of these, pathology revealed chronic bronchiolitis (n=1), non-necrotizing granulomas (n=1), peribronchial lymphocytic infiltrate (n=1) and benign non-specific inflammation (n=4).
CONCLUSIONS: Symptomatic respiratory complaints are common in CVID but objective radiographic, functional, and pathologic changes vary in terms of presentation and severity.
CLINICAL IMPLICATIONS: Patients with CVID are at increased risk for pulmonary complications and should be monitored closely.
DISCLOSURE: The following authors have nothing to disclose: Darlene Nelson, Teng Moua, David Midthun, Roshini Abraham, Robert Vassallo
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