ArticleJCI insight2019
BAFF-driven B cell hyperplasia underlies lung disease in common variable immunodeficiency.
Article in JCI insight, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 48 papers, 3 of them syntheses that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
48 citing papers in PubMed, 3 syntheses or guidelines pooled it, 82 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
- Treatment Strategies for GLILD in Common Variable Immunodeficiency: A Systematic Review.Frontiers in immunology · 2021Pooled it
- Coexistent alterations of BAFF and B-cell phenotypes in complicated CVID course.The Journal of allergy and clinical immunology · 2026Article
- BAFF as a Key Modulator of Respiratory Mucosal B Cell Immunity in Viral Infection and Mucosal Vaccination.Cells · 2026Review
- Host-Pathogen Interactions Leading to Pneumonia Pathogenesis.Clinics in chest medicine · 2026Review
- Heterozygous NFKB1 variant causes inflammatory dysregulation shaped by broader genetic context in common variable immunodeficiency.JCI insight · 2026Article
- IgA defects in CVID lead to bacterial translocation, increased serum γ-interferon, and BAFF.Journal of human immunity · 2026Article
- Lymphoproliferation in Inborn Errors of Immunity: Mechanisms, Manifestations and Clinical Management, with a Focus on ALPID.ImmunoTargets and therapy · 2026Review
- Could belimumab be a new weapon in autoimmune complicated CVID?Frontiers in immunology · 2026Article
- BAFF is a marker of hypogammaglobulinemia, neuroaxonal damage and inflammation in multiple sclerosis patients on ocrelizumab.Journal of neuroinflammation · 2025Article
- Targeting cannabinoid receptor 1 for antagonism in pro-fibrotic alveolar macrophages mitigates pulmonary fibrosis.JCI insight · 2025Article
- Pathogenic TNFRSF13B Variant in an Adult Japanese Patient with Common Variable Immunodeficiency.Internal medicine (Tokyo, Japan) · 2025Article
- When Less Could be More: Successful Treatment of Granulomatous Lymphocytic Interstitial Lung Disease with Azathioprine.European journal of case reports in internal medicine · 2025Article
- BAFF-associated granulomatous lung disease in a patient with GATA2 deficiency.The journal of allergy and clinical immunology. Global · 2024Article
- Protecting children and adults with primary antibody deficiencies against common and emergent pathogens and non-infectious complications.Clinical and experimental immunology · 2024Review
- Approach to diagnosing and managing granulomatous-lymphocytic interstitial lung disease.EClinicalMedicine · 2024Review
- Sarcoidosis versus Granulomatous and Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency: A Comparative Review.Biomedicines · 2024Review
- Elevated serum B-cell activator factor levels predict rapid progressive interstitial lung disease in anti-melanoma differentiation associated protein 5 antibody positive dermatomyositis.Orphanet journal of rare diseases · 2024Article
- The impact of immune dysregulation on the risk of malignancy in common variable immunodeficiency: insights from a multicenter study.Frontiers in immunology · 2024Article
Corrections and comments
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Authors and funding
11 authors at 3 institutions in 3 countries.
Funding
Abstract
backgroundCommon variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency and is frequently complicated by interstitial lung disease (ILD) for which etiology is unknown and therapy inadequate.
methodsMedical record review implicated B cell dysregulation in CVID ILD progression. This was further studied in blood and lung samples using culture, cytometry, ELISA, and histology. Eleven CVID ILD patients were treated with rituximab and followed for 18 months.
resultsSerum IgM increased in conjunction with ILD progression, a finding that reflected the extent of IgM production within B cell follicles in lung parenchyma. Targeting these pulmonary B cell follicles with rituximab ameliorated CVID ILD, but disease recurred in association with IgM elevation. Searching for a stimulus of this pulmonary B cell hyperplasia, we found B cell-activating factor (BAFF) increased in blood and lungs of progressive and post-rituximab CVID ILD patients and detected elevation of BAFF-producing monocytes in progressive ILD. This elevated BAFF interacts with naive B cells, as they are the predominant subset in progressive CVID ILD, expressing BAFF receptor (BAFF-R) within pulmonary B cell follicles and blood to promote Bcl-2 expression. Antiapoptotic Bcl-2 was linked with exclusion of apoptosis from B cell follicles in CVID ILD and increased survival of naive CVID B cells cultured with BAFF.
conclusionCVID ILD is driven by pulmonary B cell hyperplasia that is reflected by serum IgM elevation, ameliorated by rituximab, and bolstered by elevated BAFF-mediated apoptosis resistance via BAFF-R.
fundingNIH, Primary Immune Deficiency Treatment Consortium, and Rare Disease Foundation.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.