Rituximab Monotherapy for Common Variable Immune Deficiency-Associated Granulomatous-Lymphocytic Interstitial Lung Disease

Chest: The Journal of Circulation, Respiration and Related Systems

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