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Rituximab for granulomatous lymphocytic interstitial lung disease in a patient with common variable immunodeficiency. Is single therapy enough?

Mauricio Arraya, Yolanda Castro, Joaquin Navarro, Elizabeth Sarmiento, Eduardo Fernandez-Cruz & Javier Carbone Campoverde

Abstract: Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) is an inflammatory pulmonary complication of common variable immunodeficiency (CVID) with distinctive patterns in the biopsy; Granulomatous Disease, Lymphocytic Interstitial Disease, Follicular Bronchiolitis and Organizing Pneumonitis. Without the proper treatment this complication will lead to important pulmonary dysfunction due to fibrosis. Although this process has been well recognized, protocols for a standardized treatment, and the proper timing for the use of immune modulation as well as the maintenance time with monoclonal antibodies are an unclear topic.

Case presentation: We present the case of a 57-year-old female patient with CVID. During the evaluation of an episode of autoimmune hemolytic anemia, she presented concomitantly radiological and histopathological features of GLILD. She was treated successfully with Rituximab monotherapy without any complications and is currently asymptomatic.

Conclusion: We conclude that the use of Rituximab as single therapy, modulates the lymphocytic infiltration of the lung parenchyma and stops the progression and organization of the damage mediated by B cells and indirectly by T cells, and it could be used as monotherapy in the proper timing of the diagnostic process.