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.
Highlights
Common variable immunodeficiency (CVID) represents a rare, heterogeneous primary immunodeficiency syndrome, which is characterized by hypogammaglobulinemia, impaired B cell differentiation, and poor or missing response to vaccination
In our patients with autoimmune cytopenia, Granulomatous and lymphocytic interstitial lung disease (GLILD) was more frequent (31% vs. 3% in nocytopenia patients), and the chance for development of GLILD was significant higher when patients suffered from cytopenia. (OR=15.00, 95% confidence interval 1.576 to 142.8, p = 0.0098)
Regarding GLILD-treatment, one patient showed no progress under IgRT, and one patient was treated sufficiently with prednisolone only
Summary
Common variable immunodeficiency (CVID) represents a rare, heterogeneous primary immunodeficiency syndrome, which is characterized by hypogammaglobulinemia, impaired B cell differentiation, and poor or missing response to vaccination. These criteria must be fulfilled in a patient older than two years and any other causes for hypogammaglobulinemia must be excluded [1, 2]. There is growing evidence that rituximab, as monotherapy or in combination with antimetabolites (azathioprine or mycophenolate mofetil), represents an effective therapy for GLILD too [7, 14 - 25] In this context, we want to provide our experience with three cases of CVIDpatients who received rituximab for their GLILD. Except for glucocorticoid- and immunoglobulin-administration, no standardized treatment recommendations exist
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
