Rituximab as a Single Agent for Granulomatous Lymphocytic Interstitial Lung Disease in Common Variable Immune Deficiency.

Abstract

Common variable immune deficiency (CVID) is the most common symptomatic primary immunodeficiency and is characterized by hypogammaglobulinemia that may be associated with T-cell defects. The clinical hallmarks are recurrent infections, autoimmune manifestations, and lymphoproliferation [1]. Lung involvement is common and may manifest as recurrent infection, chronic lung disease, and interstitial lung disease [1,2]. A small subset of patients with CVID develop granulomatous lymphocytic interstitial lung disease (GLILD), a restrictive interstitial lung disease characterized by a mixed pattern of granulomata and lymphocytic infiltration. Prognosis is unfavorable [2]. Recent data suggest that diagnosis of GLILD in patients CVID reduces life expectancy by 50% [3]. Studies on lung biopsy specimens from CVID patients with GLILD have shown the formation of a tertiary lymphoid structure (TLS) in the lung [4]. There is currently no consensus on a standardized treatment protocol for CVID-associated GLILD. Available data are limited to small case series and expert opinions [5]. Management to date has been with intravenous immunoglobulin, systemic corticosteroids, and combined immunosuppressive therapies, although the results have been variable [5-7]. There is growing evidence on the efficacy of the anti-CD20 monoclonal antibody rituximab in monotherapy for treatment of CVID-associated GLILD [8]. In addition, recent evidence attributes an important role to B cells in the initiation and maintenance of a TLS [8,9]. We present the case of a 37-year-old woman with CVID receiving regular treatment with subcutaneous immunoglobulins. Her clinical history was remarkable for autoimmune thyroiditis, psoriasis, and multiple allergic reactions to drugs (ie, amoxicillinclavulanate, levofloxacin, azithromycin, and contrast media for computed tomography [CT] scan). In April 2018, the patient experienced productive cough, mild fever, and dyspnea on exertion. A month later, her physical examination at a local clinic was normal, and a Manuscript received May 13, 2019; accepted for publication August 30, 2019.