Abstract

PurposePrimary antibody deficiency patients suffer from infectious and non-infectious pulmonary complications leading over time to chronic lung disease. The complexity of this pulmonary involvement poses significant challenge in differential diagnosis in patients with long life disease and increased radio sensitivity. We planned to verify the utility of chest Magnetic Resolution Imaging with Diffusion-Weighted Imaging as a radiation free technique.MethodsProspective evaluation of 18 patients with Common Variable Immunodeficiency and X-linked Agammaglobulinemia. On the same day, patients underwent Magnetic Resonance Imaging with Diffusion Weighted Imaging sequences, High Resolution Computerized Tomography and Pulmonary Function Tests, including diffusing capacity factor for carbon monoxide. Images were scored using a modified version of the Bhalla scoring system.ResultsMagnetic Resonance Imaging was non-inferior to High Resolution Computerized Tomography in the capacity to identify bronchial and parenchymal abnormalities. HRCT had a higher capacity to identify peripheral airways abnormalities, defined as an involvement of bronchial generation up to the fifth and distal (scores 2–3). Bronchial scores negatively related to pulmonary function tests. One third of consolidations and nodules had Diffusion Weighted Imaging restrictions associated with systemic granulomatous disease and systemic lymphadenopathy. Lung Magnetic Resolution Imaging detected an improvement of bronchial and parenchymal abnormalities, in recently diagnosed patients soon after starting Ig replacement.ConclusionsMagnetic Resonance Imaging with Diffusion Weighted Imaging was a reliable technique to detect lung alterations in patients with Primary Antibody Deficiencies.Electronic supplementary materialThe online version of this article (doi:10.1007/s10875-015-0172-2) contains supplementary material, which is available to authorized users.

Highlights

  • Patients with X-linked Agammaglobulinemia (XLA) and Common Variable Immunodeficiency (CVID) suffer from recurrent respiratory infections and non-infectious pulmonary diseases possibly leading, over time, to permanent lung damage [1,2,3,4]

  • Patients with primary antibody deficiencies (PAD) are affected by a group of lung diseases, J Clin Immunol (2015) 35:491–500 denominated granulomatous-lymphocytic interstitial lung disease (GLILD) [7,8,9,10,11] characterized by granulomatous and pulmonary lymphoid hyperplasia, histological patterns consisting of lymphocytic interstitial pneumonia (LIP), follicular bronchiolitis, and lymphoid hyperplasia [12]

  • Lung Abnormalities by Magnetic Resonance Imaging (MRI) Mean total score recorded by MRI was 8 (IQR 5–11)

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Summary

Introduction

Patients with X-linked Agammaglobulinemia (XLA) and Common Variable Immunodeficiency (CVID) suffer from recurrent respiratory infections and non-infectious pulmonary diseases possibly leading, over time, to permanent lung damage [1,2,3,4]. J Clin Immunol (2015) 35:491–500 denominated granulomatous-lymphocytic interstitial lung disease (GLILD) [7,8,9,10,11] characterized by granulomatous and pulmonary lymphoid hyperplasia, histological patterns consisting of lymphocytic interstitial pneumonia (LIP), follicular bronchiolitis, and lymphoid hyperplasia [12]. The complexity of this pulmonary involvement poses a significant challenge in differential diagnosis [13]. DWI has been validated as diagnostic tool in various inflammatory diseases in the abdomen, pelvis and brain [22,23,24]

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