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
1 Department of Medicine II, Division of Rheumatology and Clinical Immunology, University Hospital Wuerzburg, Wuerzburg, Germany


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Creative Commons License
© 2021 Strunz et al.

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.

* Address correspondence to this author at the Department of Medicine II, Division of Rheumatology and Clinical Immunology, University Hospital Wuerzburg, Wuerzburg, Germany; E-mail: Strunz_P@ukw.de


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.

Keywords: Rituximab, Common variable immunodeficiency, Granulomatous and lymphocytic interstitial lung disease, Central nervous system involvement, Primary immunodeficiency, Immunotherapy.