Abstract
Introduction: In the absence of robust clinical evidence and guidelines the management of GLILD (a rare complication of common variable immunodeficiency disorders, CVID) is primarily based on clinical experience. We run the largest GLILD centre in the UK and have used a treatment protocol for years. Our current first line treatment is high-dose prednisolone (up to 1mg/kg/day). Pulmonary function tests (PFT) are performed to evaluate the course of GLILD and treatment response. We assessed initial PFT responses to steroids, regarding the magnitude of change in spirometry and gas transfer to inform which parameters were affected most.
Method: Data on treatment and lung function tests were collected from 9 patients (5 males, 4 females, age 32-71y), all on immunoglobulin replacement, starting steroids for GLILD at the Respiratory Immunology service of the Royal Free Hospital, London. We assessed change in %predicted FEV1, FVC, DLCO and KCO. Comparisons were performed for the initial change with prednisolone therapy.
Results: The results are presented in the Table. The median (IQR) time between the pre- and post1-treatment tests was 1 (1-2) months and post2 2(1-4) months. Gas transfer increased in 5/9 patients suggesting that PFT responses to steroids are heterogeneous
Conclusion: Prednisolone improves lung function in GLILD but responses are heterogeneous. The greatest initial changes are observed in spirometry than gas transfer.
Reference:
Hurst JR, et al. BLF/UKPIN Consesus Statement, J Allergy Clin Immunol Pract. 2017 Jul - Aug;5(4):938-945
Footnotes
Cite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA1408.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2019