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-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

Department of Paediatrics and Adolescent Medicine

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
Journal	Arthritis Care & Research
Volume	74
Issue number	3
Pages (from-to)	364-370
Number of pages	7
ISSN	2151-464X
DOIs	https://doi.org/10.1002/acr.24499
Publication status	Published - Mar 2022

Keywords

Adolescent
Child
Female
Humans
Lung Diseases, Interstitial/complications
Male
Missed Diagnosis
Prospective Studies
ROC Curve
Scleroderma, Systemic/complications
Tomography, X-Ray Computed
Vital Capacity

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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 & Research, 74(3), 364-370. https://doi.org/10.1002/acr.24499

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 & Research, vol. 74, no. 3, pp. 364-370. https://doi.org/10.1002/acr.24499

@article{7db0f6cc331649e79ea2e6c16d34ecfd,

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 ~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.",

keywords = "Adolescent, Child, Female, Humans, Lung Diseases, Interstitial/complications, Male, Missed Diagnosis, Prospective Studies, ROC Curve, Scleroderma, Systemic/complications, Tomography, X-Ray Computed, Vital Capacity",

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 = "{\textcopyright} 2020 American College of Rheumatology.",

year = "2022",

month = mar,

doi = "10.1002/acr.24499",

language = "English",

volume = "74",

pages = "364--370",

journal = "Arthritis Care & Research",

issn = "2151-464X",

publisher = "JohnWiley & Sons, Inc",

number = "3",

}

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

Y1 - 2022/3

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.

KW - Adolescent

KW - Child

KW - Female

KW - Humans

KW - Lung Diseases, Interstitial/complications

KW - Male

KW - Missed Diagnosis

KW - Prospective Studies

KW - ROC Curve

KW - Scleroderma, Systemic/complications

KW - Tomography, X-Ray Computed

KW - Vital Capacity

UR - http://www.scopus.com/inward/record.url?scp=85125040893&partnerID=8YFLogxK

U2 - 10.1002/acr.24499

DO - 10.1002/acr.24499

M3 - Journal article

C2 - 33141441

SN - 2151-464X

VL - 74

SP - 364

EP - 370

JO - Arthritis Care & Research

JF - Arthritis Care & Research

IS - 3

ER -

Underdetection of Interstitial Lung Disease in Juvenile Systemic Sclerosis

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