Citation, DOI, disclosures and article data
Citation:
Weerakkody Y, Silverstone L, Sharma R, et al. Granulomatous lymphocytic interstitial lung disease. Reference article, Radiopaedia.org (Accessed on 13 Mar 2025) https://doi.org/10.53347/rID-64047
Granulomatous-lymphocytic interstitial lung disease (GL-ILD) describes non-infectious diffuse lung disease complications that have been reported to traditionally develop in patients with common variable immunodeficiency (CVID).
Associations
Although it is almost always associated with CVID, some papers also report the occurrence of these conditions with primary immunodeficiency states such as 6:
They exhibit both granulomatous and lymphoproliferative histologic patterns consisting of:
CT
Pulmonary findings tend to have a mid- to lower zones distribution and are usually better depicted on CT 7, including:
Splenomegaly is almost always seen in those patients.
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Treatment and prognosis
Immunosuppressive therapy (e.g. rituximab) has been successfully used in the treatment of granulomatous lymphocytic interstitial lung disease. The presence of granulomatous lymphocytic interstitial lung disease in association with common variable immunodeficiency confers a poorer prognosis and also carries an increased risk of lymphoproliferative disorders.
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1. Park JH, Levinson AI. Granulomatous-lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (CVID). (2010) Clinical immunology (Orlando, Fla.). 134 (2): 97-103. doi:10.1016/j.clim.2009.10.002 - Pubmed
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2. Rao N, Mackinnon AC, Routes JM. Granulomatous and lymphocytic interstitial lung disease: a spectrum of pulmonary histopathologic lesions in common variable immunodeficiency--histologic and immunohistochemical analyses of 16 cases. (2015) Human pathology. 46 (9): 1306-14. doi:10.1016/j.humpath.2015.05.011 - Pubmed
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3. Lieberman P, Routes J. Granulomatous-lymphocytic interstitial lung disease in a patient with common variable immunodeficiency. (2014) The journal of allergy and clinical immunology. In practice. 2 (6): 824. doi:10.1016/j.jaip.2014.09.002 - Pubmed
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4. Tashtoush B, Memarpour R, Ramirez J, Bejarano P, Mehta J. Granulomatous-lymphocytic interstitial lung disease as the first manifestation of common variable immunodeficiency. (2018) The clinical respiratory journal. 12 (1): 337-343. doi:10.1111/crj.12511 - Pubmed
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5. Pathria M, Urbine D, Zumberg MS, Guarderas J. Management of granulomatous lymphocytic interstitial lung disease in a patient with common variable immune deficiency. (2016) BMJ Case Reports. 2016: bcr2016215624. doi:10.1136/bcr-2016-215624 - Pubmed
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6. Sood AK, Funkhouser W, Handly B, Weston B, Wu EY. Granulomatous-Lymphocytic Interstitial Lung Disease in 22q11.2 Deletion Syndrome: a Case Report and Literature Review. (2018) Current allergy and asthma reports. 18 (3): 14. doi:10.1007/s11882-018-0769-7 - Pubmed
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7. Muhammad Naeem, David H. Ballard, Hamza Jawad, Constantine Raptis, Sanjeev Bhalla. Noninfectious Granulomatous Diseases of the Chest. (2020) RadioGraphics. 40 (4): 1003-1019. doi:10.1148/rg.2020190180 - Pubmed
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8. Meerburg J, Hartmann I, Goldacker S et al. Analysis of Granulomatous Lymphocytic Interstitial Lung Disease Using Two Scoring Systems for Computed Tomography Scans—A Retrospective Cohort Study. Front Immunol. 2020;11:589148. doi:10.3389/fimmu.2020.589148 - Pubmed
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9. Scarpa R, Cinetto F, Milito C et al. Common and Uncommon CT Findings in CVID-Related GL-ILD: Correlations with Clinical Parameters, Therapeutic Decisions and Potential Implications in the Differential Diagnosis. J Clin Immunol. 2023;43(8):1903-15. doi:10.1007/s10875-023-01552-1 - Pubmed
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