Chest: The Journal of Circulation, Respiration and Related Systems | |
Rituximab Monotherapy for Common Variable Immune Deficiency-Associated Granulomatous-Lymphocytic Interstitial Lung Disease | |
Kyle Wright^21  Julie Ng^12  Maura Alvarez^13  | |
[1] Department of Pathology, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA^2;Division of Pulmonary and Critical Care, Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA^1;Division of Rheumatology, Immunology, and Allergy, Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA^3 | |
关键词: B cells; common variable immunodeficiency; granulomatous lymphocytic interstitial lung disease; rituximab; tertiary lymphoid tissue; CVID; common variable immunodeficiency; GLILD; granulomatous-lymphocytic interstitial lung disease; ILD; interstitial lung disease; TLS; tertiary lymphoid structure; | |
DOI : 10.1016/j.chest.2019.01.034 | |
学科分类:呼吸医学 | |
来源: American College of Chest Physicians | |
【 摘 要 】
Patients with common variable immunodeficiency (CVID) can develop granulomatous-lymphocytic interstitial lung disease (GLILD), which is associated with increased morbidity and mortality. Treating GLILD is a significant challenge because it is rare and can be pathologically heterogeneous. Here we describe two cases of patients with CVID-associated GLILD with biopsies demonstrating loosely organized tertiary lymphoid structures (TLSs). Based on the pivotal role that B cells play in TLS initiation and maintenance, we hypothesized that using rituximab monotherapy for B-cell depletion alone would be sufficient for the disruption of the pathologic process underlying GLILD. These two cases demonstrate that adapting a strategy of B cell depletion monotherapy may be effective in TLS-associated conditions such as GLILD.
【 授权许可】
CC BY
【 预 览 】
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RO201911040014041ZK.pdf | 1706KB | download |