Under Detection of Interstitial Lung Disease in Juvenile Systemic Sclerosis (jSSc) Utilizing Pulmonary Function Tests: Results from the Juvenile Scleroderma Inception Cohort

Ivan Foeldvari¹, Bernd Hinrichs ², Kathryn Torok ³, Maria José Santos ⁴, Ozgur Kasapcopur ⁵, Amra Adrovic ⁶, Valda Stanevicha ⁷, Flavio Sztajnbok ⁸, Maria Teresa Terreri ⁹, Ekaterina Alexeeva ¹⁰, Jordi Antón ¹¹, Maria Katsicas ¹², Vanessa Smith ¹³, Tadej Avcin ¹⁴, Rolando Cimaz ¹⁵, Mikhail Kostik ¹⁶, Thomas Lehman ¹⁷, Walter Sifuentes-Giraldo ¹⁸, Simone Appenzeller ¹⁹, Mahesh Janarthanan ²⁰, Monika Moll ²¹, Dana Nemcova ²², Dieneke Schonenberg ²³, Christina Battagliotti ²⁴, Lillemor Berntson ²⁵, Blanca Bica ²⁶, Juergen Brunner ²⁷, Patricia Costa Reis ²⁸, Despina Eleftheriou ²⁹, Liora Harel ³⁰, Gerd Horneff ³¹, Tilmann Kallinich ³², Dragana Lazarevic ³³, Kirsten Minden ³⁴, Susan Nielsen ³⁵, Farzana Nuruzzaman ³⁶, Anjali Patwardhan ³⁷, Yosef Uziel ³⁸ and Nicola Helmus ¹, ¹Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany, ²Asklepios Klinik Nord - Heidberg, Hamburg, Germany, ³UPMC Children's Hospital of Pittsburgh, Pittsburgh, ⁴Rheumatology department, Hospital Garcia de Orta, Almada, Portugal, ⁵Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Istanbul, Turkey, Istanbul, Turkey, ⁶Cerrahpaşa Tıp Fakültesi, Istanbul, Turkey, ⁷Riga Stradins University, Riga, Latvia, ⁸Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, ⁹Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, ¹⁰National Medical Research Center of Children`s Health, Moscow, Russia, ¹¹Hospital Sant Joan de Déu, Barcelona, Spain, ¹²Hospital de Pediatria, Buenos Aires, Argentina, ¹³Dept. of Rheumatology, Ghent University Hospital, Ghent, Belgium; Dept. of Internal Medicine, Ghent University, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium, Gent, Belgium, ¹⁴Ljubljana University Medical Centre, Ljubljana, Slovenia, ¹⁵University Hospital Meyer, Florence, Italy, ¹⁶Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, ¹⁷Hospital for Special Surgery, New York, ¹⁸Hospital Universitario Ramón y Cajal, Madrid, Spain, ¹⁹UNICAMP Universidade Estadual de Campinas, Sao Paulo, Brazil, ²⁰Sri Ramachandra University, Chennai, India, ²¹University of Tuebingen, Tuebingen, Germany, ²²General University Hospital, Prague, Czech Republic, ²³Amsterdam UMC, Amsterdam, Netherlands, ²⁴Children's Hospital Dr. Orlando Alassia, Santa Fee, Argentina, ²⁵Uppsala University Hospital, Uppsala, Sweden, ²⁶Hospital Universitário Clementino Fraga Filho (HUCFF/UFRJ), Rio de Janeiro, Brazil, ²⁷Tirol Kliniken, Innsbruck, Austria, ²⁸Hospital de Santa Maria, Lisbon, Portugal, ²⁹Great Ormond Street Hospital, London, United Kingdom, ³⁰Tel Aviv University, Tel Aviv, Israel, ³¹Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, ³²Charite, Berlin, Germany, ³³KC Niš, Niš, Serbia, ³⁴German Rheumatism Research Center and Charité University Medicine, Berlin, Germany, ³⁵Department of Pediatrics, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., Copenhavn, Denmark, ³⁶Stony Brook Children's Hospital, Stony Brook, NY, ³⁷University of Missouri, Columbia, ³⁸Meir Medical Center, Kfar Saba, Israel

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: interstitial lung disease, juvenile sclerosis and Pulmonary Involvement

Session Information

Date: Sunday, November 10, 2019

Title: Pediatric Rheumatology – ePoster I: Basic Science, Biomarkers, & Sclerodermic Fever

Session Type: Poster Session (Sunday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Juvenile systemic sclerosis(jSSc) is an orphan disease with a prevalence in around 3 in a million children
[1]
. Pulmonary involvement in jSSc occurs in approximately 40 % in the inception cohort [2]. Traditionally in jSSc, pulmonary function testing (PFT) with FVC and DLCO are used for screening and computed tomography (HRCT) was more reserved for those with abnormal PFTs. More recently, it has become apparent that PFTs might not be sensitive enough for detecting ILD in children.

Methods: Utilizing a prospective international juvenile systemic scleroderma cohort (JSScC) [2], to determine if pulmonary screening with FVC and DLCO is sufficient enough to assess the presence of interstitial lung disease in comparison to CT evaluation.

The JSScC cohort database was queried for available patients with recorded PFT parameters and HRCT performed to determine sensitivity of PFTs detecting disease process.

Results: Of 129 patients in the jSScC, 67 patients had both CT imaging and an FVC reading from PFTs for direct comparison. DLCO readings were also captured but not in as many patients with tandem HRCT (n =55 DCLO and HRCT scan). Therefore, initial analyses focused on the sensitivity, specificity and accuracy of the FVC value from the PFTs to capture the diagnosis of interstitial lung disease as determined by HRCT. Table 1 presents these diagnostic test evaluations for the FVC. Overall, 49% of the patients had ILD determined by HRCT, with 60% of patients having normal FVC ( >80%) with positive HRCT findings, and 24% of patients having normal DLCO ( > 80%) with positive HRCT findings. Fourteen percent (n = 3/21) of patients with both FVC and DLCO values within the normal range had a positive HRCT finding.

Conclusion: The sensitivity of the FVC in the JSScC cohort in detecting ILD was only 39%. Relying on PFTs alone for screening for ILD in juvenile systemic sclerosis would have missed the detection of ILD in almost 2/3 of the sample cohort, supporting the use of HRCT for detection of ILD in children with SSc. In addition, the cut off utilized, of less than 80% of predicted FVC or DLCO could be too low for pediatric patients to exclude beginning ILD. This pilot data needs confirmation in a larger patient population.

Supported by the “Joachim Herz Stiftung”

Beukelman, T., F. Xie, and I. Foeldvari, Assessing the prevalence of juvenile systemic sclerosis in childhood using administrative claims data from the United States.Journal of Scleroderma and Related Disorders, 2018. 3(2): p. 189-190.
Foeldvari, I., et al., CHARACTERISTICS OF THE FIRST 80 PATIENTS AT TIMEPOINT OF FIRST ASSESSMENT INCLUDED IN THE JUVENILE SYSTEMIC SCLEROSIS INCEPTION COHORT. WWW.JUVENILESCLERODERMA.COM.Journal of Scleroderma and Related Disorders, 2019. 4: p. 49-61.

ACR 2019 jssc ILD table

Disclosure: I. Foeldvari, Beyer, 5, BMS, 5, Glaxo, 5, Inventa, 5, Novartis, 5; B. Hinrichs, None; K. Torok, None; M. Santos, AbbVie, 8, Biogen, 8, Novartis, 8, Pfizer, 8, Roche, 8; O. Kasapcopur, AbbVie, 2; A. Adrovic, None; V. Stanevicha, None; F. Sztajnbok, None; M. Terreri, None; E. Alexeeva, None; J. Antón, AbbVie, 2, Bristol-Myers Squibb, 2, Gebro, 2, GlaxoSmithKline, 2, Novartis, 2, Novimmune, 2, Pfizer, 2, Roche, 2, Sobi, 2; M. Katsicas, None; V. Smith, None; T. Avcin, None; R. Cimaz, None; M. Kostik, None; T. Lehman, None; W. Sifuentes-Giraldo, None; S. Appenzeller, None; M. Janarthanan, None; M. Moll, None; D. Nemcova, None; D. Schonenberg, None; C. Battagliotti, None; L. Berntson, None; B. Bica, None; J. Brunner, None; P. Costa Reis, None; D. Eleftheriou, None; L. Harel, None; G. Horneff, Chugai, 5, 8, GlaxoSmithKline, 5, 8, Novartis, 5, 8, Sanofi, 5, 8; T. Kallinich, None; D. Lazarevic, None; K. Minden, AbbVie, 8, Abbvie, 8, German Arthritis Foundation, 2, GlaxoSmithKline, 5, GSK, 5, Sanofi, 8; S. Nielsen, None; F. Nuruzzaman, None; A. Patwardhan, None; Y. Uziel, None; N. Helmus, None.

To cite this abstract in AMA style:

Foeldvari I, Hinrichs B, Torok K, Santos M, Kasapcopur O, Adrovic A, Stanevicha V, Sztajnbok F, Terreri M, Alexeeva E, Antón J, Katsicas M, Smith V, Avcin T, Cimaz R, Kostik M, Lehman T, Sifuentes-Giraldo W, Appenzeller S, Janarthanan M, Moll M, Nemcova D, Schonenberg D, Battagliotti C, Berntson L, Bica B, Brunner J, Costa Reis P, Eleftheriou D, Harel L, Horneff G, Kallinich T, Lazarevic D, Minden K, Nielsen S, Nuruzzaman F, Patwardhan A, Uziel Y, Helmus N. Under Detection of Interstitial Lung Disease in Juvenile Systemic Sclerosis (jSSc) Utilizing Pulmonary Function Tests: Results from the Juvenile Scleroderma Inception Cohort [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/under-detection-of-interstitial-lung-disease-in-juvenile-systemic-sclerosis-jssc-utilizing-pulmonary-function-tests-results-from-the-juvenile-scleroderma-inception-cohort/. Accessed .

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