ArticleThe journal of allergy and clinical immunology. In practice
Progression of Common Variable Immunodeficiency Interstitial Lung Disease Accompanies Distinct Pulmonary and Laboratory Findings.
Article in The journal of allergy and clinical immunology. In practice. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers, 2 of them syntheses that pooled it.
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Who cites it
36 citing papers in PubMed, 2 syntheses or guidelines pooled it, 67 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
- Characteristics of severe disease in granulomatous lymphocytic interstitial lung disease: a retrospective cohort study.ERJ open research · 2026Article
- IgA defects in CVID lead to bacterial translocation, increased serum γ-interferon, and BAFF.Journal of human immunity · 2026Article
- The common variable immunodeficiency IgM repertoire narrowly recognizes erythrocyte and platelet glycans.The Journal of allergy and clinical immunology · 2024Article
- Clinical and experimental treatment of primary humoral immunodeficiencies.Clinical and experimental immunology · 2024Review
- Article
- Soluble Interleukin-2 Receptor/White Blood Cell Ratio Reflects Granulomatous Disease Progression in Common Variable Immune Deficiency.Journal of clinical immunology · 2023Article
- Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients.Journal of clinical immunology · 2023Article
- Dysregulated Lymphocyte Antigen Receptor Signaling in Common Variable Immunodeficiency with Granulomatous Lymphocytic Interstitial Lung Disease.Journal of clinical immunology · 2023Article
- Raised Serum Markers of T Cell Activation and Exhaustion in Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency.Journal of clinical immunology · 2022Article
- Article
- Pulmonary granulomatosis of genetic origin.European respiratory review : an official journal of the European Respiratory Society · 2021Review
- Interstitial Lung Disease in Common Variable Immunodeficiency.Frontiers in immunology · 2021Article
- Chronic Lung Disease in Primary Antibody Deficiency: Diagnosis and Management.Immunology and allergy clinics of North America · 2020Review
- Granulomatous lymphocytic interstitial lung disease: limiting immunosuppressive therapy-a single-centre experience.Respirology case reports · 2020Article
- Immunological and Clinical Phenotyping in Primary Antibody Deficiencies: a Growing Disease Spectrum.Journal of clinical immunology · 2020Article
- Article
- Managing Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders: e-GLILDnet International Clinicians Survey.Frontiers in immunology · 2020Article
- Analysis of Granulomatous Lymphocytic Interstitial Lung Disease Using Two Scoring Systems for Computed Tomography Scans-A Retrospective Cohort Study.Frontiers in immunology · 2020Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
backgroundCommon variable immunodeficiency may be complicated by interstitial lung disease, which leads to worsened morbidity and mortality in some. Although immunomodulatory treatment has efficacy, choice of patient, duration of treatment, and long-term follow-up are not available. Interstitial lung disease appears stable in certain instances, so it is not known whether all patients will develop progressive disease or require immunomodulatory therapy.
objectiveThis study aims to determine if all common variable immunodeficiency patients with interstitial lung disease have physiological worsening, and if clinical and/or laboratory parameters may correlate with disease progression.
methodsA retrospective review of medical records at Mount Sinai Medical Center in New York was conducted for referred patients with common variable immunodeficiency, CT scan-confirmed interstitial lung disease, and periodic pulmonary function testing covering 20 or more months before immunomodulatory therapy. Fifteen patients were identified from the retrospective review and included in this study.
resultsOf the 15 patients with common variable immunodeficiency, 9 had physiological worsening of interstitial lung disease adapted from consensus guidelines, associated with significant reductions in forced expiratory volume in 1 second, forced vital capacity, and diffusion capacity of the lung for carbon monoxide. Those with progressive lung disease also had significantly lower mean immunoglobulin G levels, greater increases and highest levels of serum immunoglobulin M (IgM), and more significant thrombocytopenia.
conclusionInterstitial lung disease resulted in physiological worsening in many, but not all subjects, and was associated with suboptimal immunoglobulin G replacement. Those with worsening pulmonary function tests, elevated IgM, and severe thrombocytopenic episodes appear to be at highest risk for progressive disease. Such patients may benefit from immunomodulatory treatment.
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