ArticleJournal of clinical immunology2019
Factors Beyond Lack of Antibody Govern Pulmonary Complications in Primary Antibody Deficiency.
Article in Journal of clinical immunology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 39 citations in OpenAlex.
- Demographic, clinical, and immunological features in combined immunodeficiency patients: a comparative analysis of those with and without pulmonary manifestations - a multicenter study from Iran.BMC pulmonary medicine · 2026Article
- Nutritional status in pediatric patients with predominant antibody deficiencyBiomedica : revista del Instituto Nacional de Salud · 2024Article
- Multiple-breath washout to detect lung disease in patients with inborn errors of immunity.ERJ open research · 2024Article
- IgDPathogens (Basel, Switzerland) · 2024Article
- Respiratory Comorbidities Associated with Bronchiectasis in Patients with Common Variable Immunodeficiency in the USIDNET Registry.Journal of clinical immunology · 2023Article
- Current Issues in the Management of IgG Subclass Deficiencies in Adults With Chronic Respiratory Diseases.Allergy, asthma & immunology research · 2023Review
- Interstitial Lung Disease in Immunocompromised Children.Diagnostics (Basel, Switzerland) · 2022Article
- Clinical and genetic findings in two siblings with X-Linked agammaglobulinemia and bronchiolitis obliterans: a case report.BMC pediatrics · 2022Article
- The pediatric common variable immunodeficiency - from genetics to therapy: a review.European journal of pediatrics · 2022Review
- Comparison of pulmonary lesions using lung ultrasound and high-resolution computed tomography in adult patients with primary humoral immunodeficiencies.Frontiers in immunology · 2022Article
- Convergence of cytokine dysregulation and antibody deficiency in common variable immunodeficiency with inflammatory complications.The Journal of allergy and clinical immunology · 2022Article
- Lymphoid malignancy in common variable immunodeficiency in a single-center cohort.European journal of haematology · 2021Article
- Clinical disparity of primary antibody deficiency patients at a safety net hospital.The journal of allergy and clinical immunology. In practice · 2021Article
- State-of-the-art diagnostic evaluation of common variable immunodeficiency.Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology · 2021Review
- Interstitial Lung Disease in Common Variable Immunodeficiency.Frontiers in immunology · 2021Article
- Shared Pathogenetic Features Between Common Variable Immunodeficiency and Sjögren's Syndrome: Clues for a Personalized Medicine.Frontiers in immunology · 2021Review
- Chronic Lung Disease in Primary Antibody Deficiency: Diagnosis and Management.Immunology and allergy clinics of North America · 2020Review
- The Importance of Primary Immune Deficiency Registries: The United States Immunodeficiency Network Registry.Immunology and allergy clinics of North America · 2020Review
- Pulmonary Disease Burden in Primary Immune Deficiency Disorders: Data from USIDNET Registry.Journal of clinical immunology · 2020Article
- B Cell Dysregulation in Common Variable Immunodeficiency Interstitial Lung Disease.Frontiers in immunology · 2020Review
Corrections and comments
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Authors and funding
4 authors at 4 institutions in 1 country.
Funding
Abstract
purposePulmonary complications occur frequently in primary antibody deficiency (PAD). While the impact of antibody deficiency may appear implicit for certain respiratory infections, immunoglobulin replacement therapy does not completely ameliorate pulmonary complications in PAD. Thus, there may be antibody-independent factors influencing susceptibility to respiratory disease in PAD, but these remain incompletely defined.
methodsWe harnessed the multicenter US Immunodeficiency Network primary immunodeficiency registry to compare prevalence of asthma, bronchiectasis, interstitial lung disease (ILD), and respiratory infections between two forms of PAD: common variable immunodeficiency (CVID) and x-linked agammaglobulinemia (XLA). We also defined the clinical and immunological characteristics associated with ILD and asthma in CVID.
resultsAsthma, bronchiectasis, ILD, pneumonia, and upper respiratory infections were more prevalent in CVID than XLA. ILD was associated with autoimmunity, bronchiectasis, and pneumonia as well as fewer B and T cells in CVID. Asthma was the most common chronic pulmonary complication and associated with lower IgA and IgM in CVID. Age of symptom onset or CVID diagnosis was unrelated with ILD or asthma.
conclusionDespite having less severe immunoglobulin deficiency than XLA, respiratory infections, ILD, and asthma were more common in CVID. Among CVID patients, ILD was associated with autoimmunity and reduced lymphocytes and asthma with lower immunoglobulins. Though our results are tempered by registry limitations, they provide evidence that factors beyond lack of antibody promote pulmonary complications in PAD. Efforts to understand how genetic etiology, nature of concurrent T cell deficiency, and propensity for autoimmunity shape pulmonary disease may improve treatment of PAD.
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