RESEARCH ARTICLE
Rituximab for the Treatment of Common Variable Immunodeficiency (CVID) with Pulmonary and Central Nervous System Involvement
Patrick-Pascal Strunz1, *, Matthias Fröhlich1, Michael Gernert1, Eva C. Schwaneck1, Lea-Kristin Nagler1, Anja Kroiss1, Hans-Peter Tony1, Marc Schmalzing1
Article Information
Identifiers and Pagination:
Year: 2021Volume: 15
First Page: 9
Last Page: 15
Publisher ID: TORJ-15-9
DOI: 10.2174/1874312902115010009
Article History:
Received Date: 7/9/2020Revision Received Date: 13/1/2021
Acceptance Date: 28/1/2021
Electronic publication date: 12/04/2021
Collection year: 2021
open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: (https://creativecommons.org/licenses/by/4.0/legalcode). This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Abstract
Background:
Granulomatous and lymphocytic interstitial lung disease (GLILD) represents a typical form of pulmonary manifestation of CVID. Except for glucocorticoid- and immunoglobulin-administration, no standardized treatment recommendations exist.
Objective:
To investigate our CVID-patients with GLILD for the applied immunosuppressive regimen, with a focus on rituximab.
Methods:
A retrospective analysis of all CVID-patients for the manifestation and treatment of GLILD at a single German center was performed in this study. For the evaluation of treatment-response, CT-imaging and pulmonary function testing were used.
Results:
50 patients were identified for the diagnosis of a CVID. 12% (n = 6) have radiological and/or histological confirmed diagnosis of a GLILD. Three patients received rituximab in a dose of 2 x 1000mg, separated by 2 weeks repeatedly. All patients showed radiological response and stabilization or improvement of the pulmonary function. Rituximab was used in one patient over 13 years with repeated treatment-response. Furthermore, the synchronic central nervous system-involvement of a GLILD-patient also responded to rituximab-treatment. With sufficient immunoglobulin-replacement-therapy, the occurring infections were manageable without the necessity of intensive care treatment.
Conclusion:
Rituximab might be considered as an effective and relatively safe treatment for CVID-patients with GLILD.