Underdetection of Interstitial Lung Disease in Juvenile Systemic Sclerosis

Ivan Foeldvari; 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-Giraldo; Ekaterina 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

doi:10.1002/acr.24499

Underdetection of Interstitial Lung Disease in Juvenile Systemic Sclerosis

Ivan Foeldvari^*
, 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-Giraldo

Ekaterina 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^*

^*Corresponding author for this work

Schön Klinik Hamburg Eilbek
German Rheumatism Research Center
Asklepios Klinik St. Georg
Istanbul University
Universidade do Estado do Rio de Janeiro
Universidade Federal de São Paulo
SJD Barcelona Children's Hospital
Ghent University
Hospital de Pediatría Prof. Dr. Juan P. Garrahan
Saint Petersburg State Pediatric Medical University
Yeshiva University
University of Ljubljana
University of Toronto
Sri Ramachandra Institute of Higher Education and Research
Hospital Garcia de Orta
Sir Ganga Ram Hospital
Hospital Ramon y Cajal
Russian Ministry of Health
Universidade Estadual de Campinas
Hospital de Niños "Dr. Orlando Alassia"
Uppsala University
Universidade Federal do Rio de Janeiro
University of Lisbon
NIHR Great Ormond Street Biomedical Research Centre, London, UK
Charité – Universitätsmedizin Berlin
Hospital for Special Surgery - New York
Azienda Ospedaliero Universitaria Meyer
University of Copenhagen
Stony Brook University
University of Missouri
SRCC Children’s Hospital
Riga Stradins University
Tel Aviv University
University of Pittsburgh
German Rheumatism Research Centrer
St.-Petersburg Pediatric Medical Academy
Montefiore Health System
Hospital for Sick Children University of Toronto
Amsterdam University Medical Centers
University College London
DANBIO

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Abstract

Objective: Utilizing data obtained from a prospective, international, juvenile systemic sclerosis (SSc) cohort, the present study was undertaken to determine if pulmonary screening with forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLco) is sufficient to assess the presence of interstitial lung disease (ILD) in comparison to high-resolution computed tomography (HRCT) in juvenile SSc. Methods: The juvenile SSc cohort database was queried for patients enrolled from January 2008 to January 2020 with recorded pulmonary function tests (PFTs) parameters and HRCT to determine the discriminatory properties of PFT parameters, FVC, and DLco in detecting ILD. Results: Eighty-six juvenile SSc patients had both computed tomography 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 juvenile SSc was only 40%, the specificity was 77%, and area under the curve (AUC) was 0.58. Fifty-eight juvenile SSc 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 juvenile SSc 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 ~60% of children who had ILD changes on their accompanying HRCT. The DLco was more sensitive in detecting potential abnormalities on 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 juvenile SSc.

Original language	English
Pages (from-to)	364-370
Number of pages	7
Journal	Arthritis care and research
Volume	74
Issue number	3
DOIs	https://doi.org/10.1002/acr.24499
Publication status	Published - 1 Mar 2022

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Foeldvari, I., Klotsche, J., Hinrichs, B., Helmus, N., Kasapcopur, O., Adrovic, A., Sztajnbok, F., Terreri, M. T., Anton, J., Smith, V., Katsicas, M., Kostik, M., Vasquez-Canizares, N., Avcin, T., Feldman, B., Janarthanan, M., Santos, M. J., Sawhney, S., Schonenberg-Meinema, D., ... Torok, K. S. (2022). Underdetection of Interstitial Lung Disease in Juvenile Systemic Sclerosis. Arthritis care and research, 74(3), 364-370. https://doi.org/10.1002/acr.24499

@article{7a97e37d859e478ebca474be597eb000,

title = "Underdetection of Interstitial Lung Disease in Juvenile Systemic Sclerosis",

abstract = "Objective: Utilizing data obtained from a prospective, international, juvenile systemic sclerosis (SSc) cohort, the present study was undertaken to determine if pulmonary screening with forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLco) is sufficient to assess the presence of interstitial lung disease (ILD) in comparison to high-resolution computed tomography (HRCT) in juvenile SSc. Methods: The juvenile SSc cohort database was queried for patients enrolled from January 2008 to January 2020 with recorded pulmonary function tests (PFTs) parameters and HRCT to determine the discriminatory properties of PFT parameters, FVC, and DLco in detecting ILD. Results: Eighty-six juvenile SSc patients had both computed tomography 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 juvenile SSc was only 40\%, the specificity was 77\%, and area under the curve (AUC) was 0.58. Fifty-eight juvenile SSc 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 juvenile SSc 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 \textasciitilde{}60\% of children who had ILD changes on their accompanying HRCT. The DLco was more sensitive in detecting potential abnormalities on 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 juvenile SSc.",

author = "Ivan Foeldvari and Jens Klotsche and Bernd Hinrichs and Nicola Helmus and Ozgur Kasapcopur and Amra Adrovic and Flavio Sztajnbok and Terreri, \{Maria Teresa\} and Jordi Anton and Vanessa Smith and Maria Katsicas and Mikhail Kostik and Natalia Vasquez-Canizares and Tadej Avcin and Brian Feldman and Mahesh Janarthanan and Santos, \{Maria Jose\} and Sujata Sawhney and Dieneke Schonenberg-Meinema and Walter-Alberto Sifuentes-Giraldo and Ekaterina Alexeeva and Simone Appenzeller and Cristina Battagliotti and Lillemor Berntson and Blanca Bica and Patr{\'i}cia Costa-Reis and Despina Eleftheriou and Tilmann Kallinich and Thomas Lehman and Edoardo Marrani and Kirsten Minden and Susan Nielsen and Farzana Nuruzzaman and Anjali Patwardhan and Raju Khubchandani and Valda Stanevicha and Yosef Uziel and Torok, \{Kathryn S.\}",

note = "Funding Information: The inception cohort project was supported by an unrestricted grant from the Joachim Herz Foundation. Publisher Copyright: {\textcopyright} 2020 American College of Rheumatology.",

year = "2022",

month = mar,

day = "1",

doi = "10.1002/acr.24499",

language = "English",

volume = "74",

pages = "364--370",

journal = "Arthritis care and research",

issn = "2151-464X",

publisher = "Elsevier USA",

number = "3",

}

Foeldvari, I, Klotsche, J, Hinrichs, B, Helmus, N, Kasapcopur, O, Adrovic, A, Sztajnbok, F, Terreri, MT, Anton, J, Smith, V, Katsicas, M, Kostik, M, Vasquez-Canizares, N, Avcin, T, Feldman, B, Janarthanan, M, Santos, MJ, Sawhney, S, Schonenberg-Meinema, D, Sifuentes-Giraldo, W-A, Alexeeva, E, Appenzeller, S, Battagliotti, C, Berntson, L, Bica, B, Costa-Reis, P, Eleftheriou, D, Kallinich, T, Lehman, T, Marrani, E, Minden, K, Nielsen, S, Nuruzzaman, F, Patwardhan, A, Khubchandani, R, Stanevicha, V, Uziel, Y & Torok, KS 2022, 'Underdetection of Interstitial Lung Disease in Juvenile Systemic Sclerosis', Arthritis care and research, vol. 74, no. 3, pp. 364-370. https://doi.org/10.1002/acr.24499

TY - JOUR

T1 - Underdetection of Interstitial Lung Disease in Juvenile Systemic Sclerosis

AU - Foeldvari, Ivan

AU - Klotsche, Jens

AU - Hinrichs, Bernd

AU - Helmus, Nicola

AU - Kasapcopur, Ozgur

AU - Adrovic, Amra

AU - Sztajnbok, Flavio

AU - Terreri, Maria Teresa

AU - Anton, Jordi

AU - Smith, Vanessa

AU - Katsicas, Maria

AU - Kostik, Mikhail

AU - Vasquez-Canizares, Natalia

AU - Avcin, Tadej

AU - Feldman, Brian

AU - Janarthanan, Mahesh

AU - Santos, Maria Jose

AU - Sawhney, Sujata

AU - Schonenberg-Meinema, Dieneke

AU - Sifuentes-Giraldo, Walter-Alberto

AU - Alexeeva, Ekaterina

AU - Appenzeller, Simone

AU - Battagliotti, Cristina

AU - Berntson, Lillemor

AU - Bica, Blanca

AU - Costa-Reis, Patrícia

AU - Eleftheriou, Despina

AU - Kallinich, Tilmann

AU - Lehman, Thomas

AU - Marrani, Edoardo

AU - Minden, Kirsten

AU - Nielsen, Susan

AU - Nuruzzaman, Farzana

AU - Patwardhan, Anjali

AU - Khubchandani, Raju

AU - Stanevicha, Valda

AU - Uziel, Yosef

AU - Torok, Kathryn S.

PY - 2022/3/1

Y1 - 2022/3/1

N2 - Objective: Utilizing data obtained from a prospective, international, juvenile systemic sclerosis (SSc) cohort, the present study was undertaken to determine if pulmonary screening with forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLco) is sufficient to assess the presence of interstitial lung disease (ILD) in comparison to high-resolution computed tomography (HRCT) in juvenile SSc. Methods: The juvenile SSc cohort database was queried for patients enrolled from January 2008 to January 2020 with recorded pulmonary function tests (PFTs) parameters and HRCT to determine the discriminatory properties of PFT parameters, FVC, and DLco in detecting ILD. Results: Eighty-six juvenile SSc patients had both computed tomography 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 juvenile SSc was only 40%, the specificity was 77%, and area under the curve (AUC) was 0.58. Fifty-eight juvenile SSc 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 juvenile SSc 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 ~60% of children who had ILD changes on their accompanying HRCT. The DLco was more sensitive in detecting potential abnormalities on 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 juvenile SSc.

AB - Objective: Utilizing data obtained from a prospective, international, juvenile systemic sclerosis (SSc) cohort, the present study was undertaken to determine if pulmonary screening with forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLco) is sufficient to assess the presence of interstitial lung disease (ILD) in comparison to high-resolution computed tomography (HRCT) in juvenile SSc. Methods: The juvenile SSc cohort database was queried for patients enrolled from January 2008 to January 2020 with recorded pulmonary function tests (PFTs) parameters and HRCT to determine the discriminatory properties of PFT parameters, FVC, and DLco in detecting ILD. Results: Eighty-six juvenile SSc patients had both computed tomography 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 juvenile SSc was only 40%, the specificity was 77%, and area under the curve (AUC) was 0.58. Fifty-eight juvenile SSc 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 juvenile SSc 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 ~60% of children who had ILD changes on their accompanying HRCT. The DLco was more sensitive in detecting potential abnormalities on 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 juvenile SSc.

UR - https://www.scopus.com/pages/publications/85125040893

U2 - 10.1002/acr.24499

DO - 10.1002/acr.24499

M3 - Article

C2 - 33141441

SN - 2151-464X

VL - 74

SP - 364

EP - 370

JO - Arthritis care and research

JF - Arthritis care and research

IS - 3

ER -

Underdetection of Interstitial Lung Disease in Juvenile Systemic Sclerosis

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