ArticleFrontiers in immunology2021
Interstitial Lung Disease in Common Variable Immunodeficiency.
Article in Frontiers in immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 2 of them syntheses that pooled it.
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Who cites it
13 citing papers in PubMed, 2 syntheses or guidelines pooled it, 24 citations in OpenAlex.
- Clinical practice guideline for the diagnosis of granulomatous-lymphocytic interstitial lung disease in patients with common variable immunodeficiency disorders: an ERS Clinical Research Collaboration.The European respiratory journal · 2026Guideline
- Diagnostic testing for interstitial lung disease in common variable immunodeficiency: a systematic review.Frontiers in immunology · 2023Pooled it
- 25-Hydroxyvitamin D Sufficiency Is Associated With Reduced Atelectasis in Common Variable Immunodeficiency: A Cross-Sectional Study.Medical science monitor : international medical journal of experimental and clinical research · 2026Article
- A systematic literature review of CVID reveals pervasive detrimental noninfectious manifestations.Journal of human immunity · 2026Article
- The Burden of Non-Infectious Organ-Specific Immunopathology in Pediatric Common Variable Immunodeficiency.International journal of molecular sciences · 2025Review
- Interstitial lung diseases (ILD) in common variable immunodeficiency (CVID) patients: a study from Iran.BMC immunology · 2024Article
- Proof-of-concept study evaluating humoral primary immunodeficiencies via CJ:KREC ratio and serum BAFF level.Scientific reports · 2024Article
- Primary immunodeficiency diseases of adults: a review of pulmonary complication imaging findings.European radiology · 2024Review
- Respiratory Comorbidities Associated with Bronchiectasis in Patients with Common Variable Immunodeficiency in the USIDNET Registry.Journal of clinical immunology · 2023Article
- ERS International Congress 2022: highlights from the Thoracic Surgery and Lung Transplantation Assembly.ERJ open research · 2023Review
- Non-infectious Pulmonary Complications in Children with Primary Immunodeficiency.Clinical medicine insights. Pediatrics · 2023Review
- Interstitial Lung Disease in Immunocompromised Children.Diagnostics (Basel, Switzerland) · 2022Article
- Diagnostic Barriers in Children with Immunodeficiencies in Central Asia: A Case-Based Discussion.Pediatric reports · 2021Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
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.
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Registered trials
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