Rituximab for the Treatment of Common Variable Immunodeficiency (CVID) with Pulmonary and Central Nervous System Involvement

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.