Under detection of interstitial lung disease in juvenile systemic sclerosis (jSSc)

Ivan Foeldvari (Coresponding Author), Jens Klotsche, Bernd Hinrichs, Nicola Helmus, Ozgur Kasapcopur, Amra Adrovic, Flavio Sztajnbok, Maria Teresa Terreri, Jordi Anton, Vanessa Smith, Maria Katsicas, Mikhail Kostik, Natalia Vasquez‐Canizares, Tadej Avcin, Brian Feldman, Mahesh Janarthanan, Maria Jose Santos, Sujata Sawhney, Dieneke Schonenberg‐Meinema, Walter‐Alberto Sifuentes‐GiraldoEkaterina Alexeeva, Simone Appenzeller, Cristina Battagliotti, Lillemor Berntson, Blanca Bica, Patrícia Costa Reis, Despina Eleftheriou, Tilmann Kallinich, Thomas Lehman, Edoardo Marrani, Kirsten Minden, Susan Nielsen, Farzana Nuruzzaman, Anjali Patwardhan, Raju Khubchandani, Valda Stanevicha, Yosef Uziel, Kathryn S. Torok

Research output: Contribution to journalArticlepeer-review


OBJECTIVES: Utilizing data obtained from a prospective international juvenile systemic sclerosis cohort (jSScC) to determine if pulmonary screening with forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (DLCO) is sufficient to assess the presence of interstitial lung disease (ILD) in comparison to high resolution computed tomography (HRCT) in jSSc.

METHODS: The jSScC cohort database was queried for patients enrolled from January 2008 to January 2020 with recorded pulmonary function tests (PFT) parameters and HRCT to determine the discriminatory properties of PFTs parameters, FVC and DLCO, in detecting ILD.

RESULTS: Eighty-six jSSc patients had both CT imaging and FVC values for direct comparison. Using findings on HRCT as the standard measure of ILD presence, the sensitivity of FVC in detecting ILD in jSSc was only 40%, the specificity was 77%, and AUC was 0.58. Fifty-eight jSSc patients had both CT imaging and DLCO values for comparison. The sensitivity of DLCO in detecting ILD was 76%, the specificity was 70%, and AUC was 0.73.

CONCLUSION: The performance of PFTs in jSSc to detect underlying ILD was quite limited. Specifically, the FVC, which is one of the main clinical parameters in adult SSc to detect and monitor ILD, would miss approximately 60% of children that had ILD changes on their accompanying HRCT. The DLCO was more sensitive in detecting potential abnormalities in HRCT, but with less specificity than the FVC. These results support the use of HRCT in tandem with PFTs for the screening of ILD in jSSc.
Original languageEnglish
Pages (from-to)1-24
JournalArthritis Care and Research
Publication statusPublished - 3 Nov 2020

Field of Science

  • 3.2 Clinical medicine

Publication Type

  • 1.1. Scientific article indexed in Web of Science and/or Scopus database


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