Use of Combination Chemotherapy for Treatment of Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD) in Patients with Common Variable Immunodeficiency (CVID)

• Published in: Journal of clinical immunology Vol. 33; no. 1; pp. 30 - 39
• Main Authors: Chase, Nicole M., Verbsky, James W., Hintermeyer, Mary K., Waukau, Jill K., Tomita-Mitchell, Aoy, Casper, James T., Singh, Sumit, Shahir, Kaushik S., Tisol, William B., Nugent, Melodee L., Rao, R. Nagarjun, Mackinnon, A. Craig, Goodman, Lawrence R., Simpson, Pippa M., Routes, John M.
• Format: Journal Article
• Language: English
• Published: Boston Springer US 01-01-2013; Springer Nature B.V
• Subjects: Administration, Oral; Adolescent; Adult; Antibodies, Monoclonal, Murine-Derived - administration & dosage; Azathioprine - administration & dosage; B-Lymphocyte Subsets - drug effects; B-Lymphocyte Subsets - immunology; B-Lymphocyte Subsets - pathology; Biomedical and Life Sciences; Biomedicine; Common Variable Immunodeficiency - drug therapy; Common Variable Immunodeficiency - immunology; Common Variable Immunodeficiency - pathology; Drug Therapy, Combination; Female; Granuloma - drug therapy; Granuloma - immunology; Granuloma - pathology; Humans; Immunology; Infectious Diseases; Infusions, Intravenous; Internal Medicine; Lung Diseases, Interstitial - drug therapy; Lung Diseases, Interstitial - immunology; Lung Diseases, Interstitial - pathology; Male; Medical Microbiology; Original Research; Retrospective Studies; Rituximab; T-Lymphocyte Subsets - drug effects; T-Lymphocyte Subsets - immunology; T-Lymphocyte Subsets - pathology; Young Adult; primary immunodeficiency; azathioprine; lung disease; Common variable immunodeficiency (CVID); rituximab; granulomatous and lymphocytic interstitial lung disease (GLILD)
• ISSN: 0271-9142; 1573-2592
• DOI: 10.1007/s10875-012-9755-3

Purpose
A subset of patients with common variable immunodeficiency (CVID) develops granulomatous and lymphocytic interstitial lung disease (GLILD), a restrictive lung disease associated with early mortality. The optimal therapy for GLILD is unknown. This study was undertaken to see if rituximab and azathioprine (combination chemotherapy) would improve pulmonary function and/or radiographic abnormalities in patients with CVID and GLILD.
Methods
A retrospective chart review of patients with CVID and GLILD who were treated with combination chemotherapy was performed. Complete pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans of the chest were done prior to therapy and >6 months later. HRCT scans of the chest were blinded, randomized, and scored independently (in pairs) by two radiologists. The differences between pre- and post-treatment HRCT scores and PFT parameters were analyzed.
Results
Seven patients with CVID and GLILD met inclusion criteria. Post-treatment increases were noted in both FEV1 (
p
 = 0.034) and FVC (
p
 = 0.043). HRCT scans of the chest demonstrated improvement in total score (
p
 = 0.018), pulmonary consolidations (
p
 = 0.041), ground-glass opacities (
p
 = 0.020) nodular opacities (
p
 = 0.024), and both the presence and extent of bronchial wall thickening (
p
 = 0.014, 0.026 respectively). No significant chemotherapy-related complications occurred.
Conclusions
Combination chemotherapy improved pulmonary function and decreased radiographic abnormalities in patients with CVID and GLILD.