The efficacy and safety of systemic corticosteroids as first line treatment for granulomatous lymphocytic interstitial lung disease.
Smits, Bas Goldacker, Sigune Seneviratne, Suranjith Malphettes, Marion Longhurst, Hilary Mohamed, Omar E Witt-Rautenberg, Carla Leeman, Lucy Schwaneck, Eva Raymond, Isabelle
Smits, Bas
Goldacker, Sigune
Seneviratne, Suranjith
Malphettes, Marion
Longhurst, Hilary
Mohamed, Omar E
Witt-Rautenberg, Carla
Leeman, Lucy
Schwaneck, Eva
Raymond, Isabelle
Citations
Altmetric:
Affiliation
Other Contributors
Publication date
2022-12-29
Subject
Collections
Research Projects
Organizational Units
Journal Issue
Abstract
Background: Granulomatous and lymphocytic interstitial lung disease (gl-ILD) is a major cause of morbidity and mortality among patients with common variable immunodeficiency. Corticosteroids are recommended as first-line treatment for gl-ILD, but evidence for their efficacy is lacking.
Objectives: This study analyzed the effect of high-dose corticosteroids (≥0.3 mg/kg prednisone equivalent) on gl-ILD, measured by high-resolution computed tomography (HRCT) scans, and pulmonary function test (PFT) results.
Methods: Patients who had received high-dose corticosteroids but no other immunosuppressive therapy at the time (n = 56) and who underwent repeated HRCT scanning or PFT (n = 39) during the retrospective and/or prospective phase of the Study of Interstitial Lung Disease in Primary Antibody Deficiency (STILPAD) were included in the analysis. Patients without any immunosuppressive treatment were selected as controls (n = 23). HRCT scans were blinded, randomized, and scored using the Hartman score. Differences between the baseline and follow-up HRCT scans and PFT were analyzed.
Results: Treatment with high-dose corticosteroids significantly improved HRCT scores and forced vital capacity. Carbon monoxide diffusion capacity significantly improved in both groups. Of 18 patients, for whom extended follow-up data was available, 13 achieved a long-term, maintenance therapy independent remission. All patients with relapse were retreated with corticosteroids, but only one-fifth of them responded. Two opportunistic infections were found in the corticosteroid treatment group, while overall infection rate was similar between cohorts.
Conclusions: Induction therapy with high-dose corticosteroids improved HRCT scans and PFT results of patients with gl-ILD and achieved long-term remission in 42% of patients. It was not associated with major side effects. Low-dose maintenance therapy provided no benefit and efficacy was poor in relapsing disease.
Keywords: CVID; Granulomatous and lymphocytic interstitial lung disease; Hartmann score; corticosteroids; gl-ILD; immune dysregulation; observational trial; pulmonary function tests; quality of life.
Citation
Smits, B., Goldacker, S., Seneviratne, S., Malphettes, M., Longhurst, H., Mohamed, O. E., Witt-Rautenberg, C., Leeman, L., Schwaneck, E., Raymond, I., Meghit, K., Uhlmann, A., Winterhalter, C., van Montfrans, J., Klima, M., Workman, S., Fieschi, C., Lorenzo, L., Boyle, S., Onyango-Odera, S., … Warnatz, K. (2023). The efficacy and safety of systemic corticosteroids as first line treatment for granulomatous lymphocytic interstitial lung disease. The Journal of allergy and clinical immunology, 152(2), 528–537. https://doi.org/10.1016/j.jaci.2022.12.813
Type
Article