Quantitative assessment of interstitial lung disease in Sjögren's syndrome

PLoS One. 2019 Nov 8;14(11):e0224772. doi: 10.1371/journal.pone.0224772. eCollection 2019.

Abstract

Background: Interstitial lung disease (ILD) is a frequent manifestation of Sjögren's syndrome (SS), an autoimmune disease of salivary and lacrimal glands, and affects approximately 20% of patients. No clinical or serological features appear to be useful to predict its presence, severity or progression, and chest high-resolution computed tomography (CT) remains the gold standard for diagnosis. Semiquantitative CT (SQCT) based on visual assessment (Goh and Taouli scoring) can estimate ILD extent, although it is burdened by relevant intra- and interobserver variability. Quantitative chest CT (QCT) is a promising alternative modality to assess ILD severity.

Aim: To determine whether QCT assessment can identify extensive or limited lung disease in patients with SS and ILD.

Methods: This multi-center, cross-sectional and retrospective study enrolled patients with SS and a chest CT scan. SQCT assessment was carried out in a blinded and centralized manner to calculate both Goh and Taouli scores. An operator-independent analysis of all CT scans with the open-source software platform Horos was used to evaluate the QCT indices. Patients were classified according to the extent of ILD and differences in QCT index distribution were investigated with non-parametric tests.

Results: From a total of 102 consecutive patients with SS, the prevalence of ILD was 35.3% (36/102). There was a statistically significant difference in QCT index distribution between the SS with ILD and SS without ILD groups (p<0.001). Moreover, SS-ILD patients with ILD >20% (by Goh score) had a QCT index statistically different from those with limited ILD extent (p<0.001). Finally, QCT indices showed a moderate-to-good correlation with the Goh and Taouli scores (from 0.44 to 0.65; p<0.001).

Conclusions: QCT indices can identify patients with SS and ILD and discriminate those with lesser or greater lung disease.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Female
  • Humans
  • Lung Diseases, Interstitial / complications*
  • Lung Diseases, Interstitial / diagnostic imaging
  • Lung Diseases, Interstitial / physiopathology
  • Male
  • Middle Aged
  • Respiratory Function Tests
  • Sensitivity and Specificity
  • Sjogren's Syndrome / complications*
  • Sjogren's Syndrome / diagnosis*
  • Tomography, X-Ray Computed

Associated data

  • Dryad/10.5061/dryad.sbcc2fr22

Grants and funding

This work was supported by Internal/own funds of the Complejo Hospitalario Ruber Juan Bravo for clinical research. The funder had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.