Academic Journal
Granulomatous Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency.
| Τίτλος: | Granulomatous Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency. |
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| Συγγραφείς: | Benor S; Department of Allergy and Clinical Immunology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel., Hentschel F; Department of Allergy and Clinical Immunology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel., Freund T; Department of Allergy and Clinical Immunology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel., Hagin D; Department of Allergy and Clinical Immunology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel. |
| Πηγή: | The Israel Medical Association journal : IMAJ [Isr Med Assoc J] 2026 Aug; Vol. 28 (8), pp. 500-507. |
| Τύπος έκδοσης: | Journal Article |
| Γλώσσα: | English |
| Στοιχεία περιοδικού: | Publisher: Israel Medical Association Country of Publication: Israel NLM ID: 100930740 Publication Model: Print Cited Medium: Internet ISSN: 3135-8225 (Electronic) NLM ISO Abbreviation: Isr Med Assoc J Subsets: MEDLINE |
| Imprint Name(s): | Original Publication: Ramat Gan, Israel : Israel Medical Association, c1999- |
| Ιατρικοί όροι (MeSH): | Common Variable Immunodeficiency*/complications , Common Variable Immunodeficiency*/immunology , Common Variable Immunodeficiency*/diagnosis , Lung Diseases, Interstitial*/etiology , Lung Diseases, Interstitial*/immunology , Lung Diseases, Interstitial*/diagnosis , Lung Diseases, Interstitial*/physiopathology , Granuloma*, B-Lymphocytes/immunology ; T-Lymphocytes/immunology ; Humans ; Female ; Male ; Adult ; Immunophenotyping ; Middle Aged |
| Περίληψη: | Background: Common variable immunodeficiency (CVID) is the most common clinically significant primary immunodeficiency disorder, typically presenting with hypogammaglobulinemia and recurrent infections. Mechanistically, CVID is caused by abnormal B-cell maturation or function. These abnormalities lead to an impaired ability to generate a normal quantity or diversity of antibodies and an impaired response to neo-antigens. In addition to the resulted immunodeficiency, CVID can also involve immune dysregulation affecting different organ systems, with the lungs being one of the more commonly involved organs. Granulomatous lymphocytic interstitial lung disease (GLILD) is a challenging and well known CVID-related complication. It is associated with high morbidity and increased risk of mortality. Objectives: To describe the clinical and immunological features of five CVID patients with radiologic and/or pathologic features consistent with granulomatous lymphocytic interstitial lung disease. Methods: We conducted a review of clinical, laboratory, imaging, and pathology data, as well as B-cell and T-cell immunophenotyping of patient PBMCs, focusing on unique characteristics of CVID GLILD patients. Results: CVID GLILD patients showed several clinical characteristics, including multiorgan involvement with cytopenias and lymphoproliferation but without significant gastrointestinal involvement. B-cell phenotyping showed abnormal B-cell maturation and development with severely impaired class switching. In addition, unique T-cell phenotype with low to near-absent naive CD4+ T cells was observed. Conclusions: Lung involvement in the form of GLILD is associated with significant co-morbidity and typical B-cell and T-cell immunophenotyping. Patients should be actively screened for the possibility of GLILD, especially when regularly performed lymphocyte immunophenotyping suggests similar patterns. |
| Entry Date(s): | Date Created: 20260806 Date Completed: 20260806 Latest Revision: 20260806 |
| Update Code: | 20260806 |
| PMID: | 42557667 |
| Βάση Δεδομένων: | MEDLINE |
| ISSN: | 3135-8225 |
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