title: Granulomatous-lymphocytic interstitial lung disease as the first manifestation of common variable immunodeficiency.
abstract: Common variable immunodeficiency (CVID) is one of the most common primary immunodeficiencies, which is characterized by reduced serum immunoglobulin levels and B-lymphocyte dysfunction. There are many clinical manifestations of this disease, the most common of which are recurrent respiratory tract infections. Among the most recently recognized autoimmune manifestation of CVID is a disease described as granulomatous-lymphocytic interstitial lung disease (GLILD), where CVID coexists with a small airway lymphoproliferative disorder, mimicking follicular bronchiolitis, or lymphocytic interstitial pneumonitis (LIP) on histology specimens. We herein describe the clinical and radiological features of GLILD in a 55-year-old woman where the diagnosis of CVID was actively pursued and eventually confirmed after her lung biopsy showed characteristic features of GLILD. The patient had dramatic response to treatment with IVIG and corticosteroids for 3 months followed by Mycophenolate mofetil for maintenance therapy.
authors.affiliation: Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Florida, Weston, Florida.
authors.forename: Basheer
authors.initials: B
authors.lastname: Tashtoush
authors.affiliation: Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Florida, Weston, Florida.
authors.forename: Roya
authors.initials: R
authors.lastname: Memarpour
authors.affiliation: Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Florida, Weston, Florida.
authors.forename: Jose
authors.initials: J
authors.lastname: Ramirez
authors.affiliation: Department of Pathology, Cleveland Clinic Florida, Weston, Florida.
authors.forename: Pablo
authors.initials: P
authors.lastname: Bejarano
authors.affiliation: Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Florida, Weston, Florida.
authors.forename: Jinesh
authors.initials: J
authors.lastname: Mehta
mesh_terms: D001706:Biopsy; D017074:Common Variable Immunodeficiency / Q000150:complications*; D005260:Female; D006099:Granuloma / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging*; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000209:etiology*; D008875:Middle Aged; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed
keywords: common variable immunodeficiency; follicular bronchiolitis; granulomatous-lymphocytic interstitial lung disease; lymphocytic interstitial pneumonitis; lymphoproliferative disorder