Analysis of Granulomatous Lymphocytic Interstitial Lung Disease Using Two Scoring Systems for Computed Tomography Scans-A Retrospective Cohort Study.

Jennifer J Meerburg,Ieneke J C Hartmann,Ulrich Baumann,Eleni-Rosalina Andrinopoulou,Harm A W M Tiddens,Mariette P C Kemner V/D Corput,Sigune Goldacker,Annette Uhlmann,Klaus Warnatz

doi:10.3389/fimmu.2020.589148

Abstract

BackgroundGranulomatous lymphocytic interstitial lung disease (GLILD) is present in about 20% of patients with common variable immunodeficiency disorders (CVID). GLILD is characterized by nodules, reticulation, and ground-glass opacities on CT scans. To date, large cohort studies that include sensitive CT outcome measures are lacking, and severity of structural lung disease remains unknown. The aim of this study was to introduce and compare two scoring methods to phenotype CT scans of GLILD patients.MethodsPatients were enrolled in the “Study of Interstitial Lung Disease in Primary Antibody Deficiency” (STILPAD) international cohort. Inclusion criteria were diagnosis of both CVID and GLILD, as defined by the treating immunologist and radiologist. Retrospectively collected CT scans were scored systematically with the Baumann and Hartmann methods.ResultsIn total, 356 CT scans from 138 patients were included. Cross-sectionally, 95% of patients met a radiological definition of GLILD using both methods. Bronchiectasis was present in 82% of patients. Inter-observer reproducibility (intraclass correlation coefficients) of GLILD and airway disease were 0.84 and 0.69 for the Hartmann method and 0.74 and 0.42 for the Baumann method.ConclusionsIn both the Hartmann and Baumann scoring method, the composite score GLILD was reproducible and therefore might be a valuable outcome measure in future studies. Overall, the reproducibility of the Hartmann method appears to be slightly better than that of the Baumann method. With a systematic analysis, we showed that GLILD patients suffer from extensive lung disease, including airway disease. Further validation of these scoring methods should be performed in a prospective cohort study involving routine collection of standardized CT scans.Clinical Trial Registration https://www.drks.de, identifier DRKS00000799.

Highlights

Common variable immunodeficiency disorders (CVID) are a heterogeneous group of primary antibody deficiency syndromes [1]
It was shown that CVID patients with Granulomatous lymphocytic interstitial lung disease (GLILD) (n = 13) have a markedly reduced survival rate of 50% compared to patients without GLILD (n = 56) and this finding led to a heightened clinical interest in the GLILD patient group [9]
Inclusion criteria were as follows: 1) CVID defined by criteria approved by the European Society for Immunodeficiencies and the Pan-American Group for Immunodeficiency [2], 2) age of 18 years and above, and 3) a radiological diagnosis of interstitial lung disease or granuloma on chest computed tomography (CT) scan, characterized by the presence of nodules, reticulation, or ground-glass opacities (GGO)

Summary

Introduction

Common variable immunodeficiency disorders (CVID) are a heterogeneous group of primary antibody deficiency syndromes [1]. It was shown that CVID patients with GLILD (n = 13) have a markedly reduced survival rate of 50% compared to patients without GLILD (n = 56) and this finding led to a heightened clinical interest in the GLILD patient group [9]. This interstitial lung disease can lead to clinical complaints such as reduced exercise tolerance and dyspnoea. Granulomatous lymphocytic interstitial lung disease (GLILD) is present in about 20% of patients with common variable immunodeficiency disorders (CVID). The aim of this study was to introduce and compare two scoring methods to phenotype CT scans of GLILD patients

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