title: FDG PET-CT imaging of therapeutic response in granulomatous lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (CVID).
abstract: Common variable immunodeficiency (CVID) is the most common severe adult primary immunodeficiency and is characterized by a failure to produce antibodies leading to recurrent predominantly sinopulmonary infections. Improvements in the prevention and treatment of infection with immunoglobulin replacement and antibiotics have resulted in malignancy, autoimmune, inflammatory and lymphoproliferative disorders emerging as major clinical challenges in the management of patients who have CVID. In a proportion of CVID patients, inflammation manifests as granulomas that frequently involve the lungs, lymph nodes, spleen and liver and may affect almost any organ. Granulomatous lymphocytic interstitial lung disease (GLILD) is associated with a worse outcome. Its underlying pathogenic mechanisms are poorly understood and there is limited evidence to inform how best to monitor, treat or select patients to treat. We describe the use of combined 2-[(18)F]-fluoro-2-deoxy-d-glucose positron emission tomography and computed tomography (FDG PET-CT) scanning for the assessment and monitoring of response to treatment in a patient with GLILD. This enabled a synergistic combination of functional and anatomical imaging in GLILD and demonstrated a widespread and high level of metabolic activity in the lungs and lymph nodes. Following treatment with rituximab and mycophenolate there was almost complete resolution of the previously identified high metabolic activity alongside significant normalization in lymph node size and lung architecture. The results support the view that GLILD represents one facet of a multi-systemic metabolically highly active lymphoproliferative disorder and suggests potential utility of this imaging modality in this subset of patients with CVID.
authors.affiliation: Department of Immunology, Immunodeficiency Centre for Wales, University Hospital of Wales, Cardiff, UK.
authors.forename: S
authors.initials: S
authors.lastname: Jolles
authors.affiliation: Department of Immunology, Immunodeficiency Centre for Wales, University Hospital of Wales, Cardiff, UK.
authors.forename: E
authors.initials: E
authors.lastname: Carne
authors.affiliation: Department of Respiratory Medicine, Neville Hall Hospital, Abergavenny, UK.
authors.forename: M
authors.initials: M
authors.lastname: Brouns
authors.affiliation: Department of Immunology, Immunodeficiency Centre for Wales, University Hospital of Wales, Cardiff, UK.
authors.forename: T
authors.initials: T
authors.lastname: El-Shanawany
authors.affiliation: Department of Immunology, Immunodeficiency Centre for Wales, University Hospital of Wales, Cardiff, UK.
authors.forename: P
authors.initials: P
authors.lastname: Williams
authors.affiliation: Department of Radiology, PETIC, University Hospital of Wales, Cardiff, UK.
authors.forename: C
authors.initials: C
authors.lastname: Marshall
authors.affiliation: Department of Radiology, PETIC, University Hospital of Wales, Cardiff, UK.
authors.forename: P
authors.initials: P
authors.lastname: Fielding
mesh_terms: D000328:Adult; D017074:Common Variable Immunodeficiency / Q000000981:diagnostic imaging* / Q000188:drug therapy; D005260:Female; D019788:Fluorodeoxyglucose F18; D015769:Granuloma, Respiratory Tract / Q000000981:diagnostic imaging* / Q000188:drug therapy; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging* / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging* / Q000188:drug therapy; D008214:Lymphocytes / Q000276:immunology*; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000627:therapeutic use; D049268:Positron-Emission Tomography / Q000379:methods*; D000069283:Rituximab / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed / Q000379:methods*; D016896:Treatment Outcome
chemical_list: D019788:Fluorodeoxyglucose F18; D000069283:Rituximab; D009173:Mycophenolic Acid
keywords: common variable immunodeficiency; fluorodeoxyglucose positron emission tomography; granulomatous lymphocytic interstitial lung disease; rituximab