Abstract
Interstitial lung disease (ILD) is a common complication in patients with common variable immunodeficiency (CVID) and often associated with other features, such as bronchiectasis and autoimmunity. As the ILD term encompasses different acute and chronic pulmonary conditions, the diagnosis is commonly made based on imaging features; histopathology is less frequently available. From a cohort of 637 patients with CVID followed at our center over 4 decades, we reviewed the data for 46 subjects (30 females, 16 males) who had lung biopsies with proven ILD. They had a median age at CVID diagnosis of 26 years old, with a median IgG level at diagnosis of 285.0 mg/dL with average isotype switched memory B cells of 0.5%. Lung biopsy pathology revealed granulomas in 25 patients (54.4%), lymphoid interstitial pneumonia in 13 patients (28.3%), lymphoid hyperplasia not otherwise specified in 7 patients (15.2%), cryptogenic organizing pneumonia in 7 patients (15.2%), follicular bronchitis in 4 patients (8.7%), and predominance of pulmonary fibrosis in 4 patients (8.7%). Autoimmune manifestations were common and were present in 28 (60.9%) patients. Nine patients (19.6%) died, with a median age at death of 49-years-old. Lung transplant was done in 3 of these patients (6.5%) who are no longer alive. These analyses reveal the high burden of this complication, with almost one-fifth of the group deceased in this period. Further understanding of the causes of the development and progression of ILD in CVID patients is required to define the best management for this patient population.
Highlights
Common variable immunodeficiency (CVID) is the most prevalent form of clinically-recognized primary immunodeficiency, characterized by low serum IgG levels, usually a low IgA, and often a low IgM, reduced or absent antibody responses to disease or immunizations
CVID is commonly associated with inflammatory complications, leading to chronic lung disease, generalized lymphoid hypertrophy, splenomegaly, gastrointestinal disease, and cytopenias, amongst other inflammatory manifestations [7,8,9,10,11]
Lung biopsy pathology revealed granulomatous infiltrates in 25 of the 46 patients (54.3%), lymphoid interstitial pneumonia in 13 (28.3%), lymphoid hyperplasia not otherwise specified in 7 (15.2%), cryptogenic organizing pneumonia in 7 (15.2%), follicular bronchitis in 4 (8.7%), and predominance of pulmonary fibrosis in 4 patients (8.7%)
Summary
Common variable immunodeficiency (CVID) is the most prevalent form of clinically-recognized primary immunodeficiency, characterized by low serum IgG levels, usually a low IgA, and often a low IgM, reduced or absent antibody responses to disease or immunizations. This defect leads to recurrent infections, with particular emphasis on the sinorespiratory tract [1,2,3,4,5,6]. Interstitial lung disease (ILD) is a term that encompasses a group of different acute and chronic pulmonary conditions with common clinical and physiological characteristics This condition is a common complication in patients with CVID. The diagnosis of ILD is commonly made based on clinical presentation and includes
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