Citation, DOI, disclosures and article data
Citation:
Weerakkody Y, Ferrario M, Di Muzio B, et al. Granulomatous lymphocytic interstitial lung disease. Reference article, Radiopaedia.org (Accessed on 29 Mar 2024) https://doi.org/10.53347/rID-64047
Granulomatous-lymphocytic interstitial lung disease (GL-ILD) is a relatively recent term given to describe non-infectious diffuse lung disease complications that have been reported to traditionally develop in common variable immunodeficiency (CVID) patients.
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:
- diffuse micronodules
- interlobular septal thickening
- bronchiectasis: tend to be mild
- lymphadenopathy
Splenomegaly is almost always seen in those patients.
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. Mohini Pathria, Daniel Urbine, Marc Stuart Zumberg, Juan Guarderas. 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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