Use of Combination Chemotherapy for Treatment of Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD) in Patients with Common Variable Immunodeficiency (CVID)
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.
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.
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.
Combination chemotherapy improved pulmonary function and decreased radiographic abnormalities in patients with CVID and GLILD.
KeywordsCommon variable immunodeficiency (CVID) primary immunodeficiency lung disease granulomatous and lymphocytic interstitial lung disease (GLILD) rituximab azathioprine
We would like to acknowledge and thank Dr. Mitchell Grayson for his thoughtful review of the manuscript.
This research was supported by National Institutes of Health grant R01CA122539 (www.nih.gov), and the Children’s Research Institute of the Children’s Hospital of Wisconsin (www.chw.org/display/PPF/DocID/30477/router.asp).
Conflict of interest
The authors of this manuscript declare that they have no financial conflicts of interest.
- 24.MacDermott RP. Immunomodulator therapy in Crohn’s disease. In: UpToDate. Basow DS, editor. Waltham, MA: UpToDate; 2012.Google Scholar
- 25.MacDermott RP. 6-mercaptopurine (6-MP) metabolite monitoring and TPMT testing in the treatment of inflammatory bowel disease with 6-MP or azathioprine. In: UpToDate. Basow DS, editor. Waltham, MA: UpToDate; 2012.Google Scholar
- 33.Salzer U, Bacchelli C, Buckridge S, Pan-Hammarstrom Q, Jennings S, Lougaris V, et al. Relevance of biallelic versus monoallelic TNFRSF13B mutations in distinguishing disease-causing from risk-increasing TNFRSF13B variants in antibody deficiency syndromes. Blood. 2008;113(9):1967–76.PubMedCrossRefGoogle Scholar