Use of combination chemotherapy for treatment of granulomatous and lymphocytic interstitial lung disease (GLILD) in patients with common variable immunodeficiency (CVID). J Clin Immunol 2013 Jan;33(1):30-9
Date
08/30/2012Pubmed ID
22930256Pubmed Central ID
PMC3557581DOI
10.1007/s10875-012-9755-3Scopus ID
2-s2.0-84876429921 (requires institutional sign-in at Scopus site) 171 CitationsAbstract
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.
Author List
Chase NM, Verbsky JW, Hintermeyer MK, Waukau JK, Tomita-Mitchell A, Casper JT, Singh S, Shahir KS, Tisol WB, Nugent ML, Rao RN, Mackinnon AC, Goodman LR, Simpson PM, Routes JMAuthors
Aoy Tomita Mitchell PhD Professor in the Surgery department at Medical College of WisconsinPippa M. Simpson PhD Adjunct Professor in the Pediatrics department at Medical College of Wisconsin
James Verbsky MD, PhD Professor in the Pediatrics department at Medical College of Wisconsin
MESH terms used to index this publication - Major topics in bold
Administration, OralAdolescent
Adult
Antibodies, Monoclonal, Murine-Derived
Azathioprine
B-Lymphocyte Subsets
Common Variable Immunodeficiency
Drug Therapy, Combination
Female
Granuloma
Humans
Infusions, Intravenous
Lung Diseases, Interstitial
Male
Retrospective Studies
Rituximab
T-Lymphocyte Subsets
Young Adult
