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 Hinrichs², Kathryn Torok³, Maria Jose Santos⁴, Ozgur Kasapcopur⁵, Amra Adrovic⁶, Valda Stanevicha⁷, Flavio Sztajnbok⁸, Maria Teresa Terreri⁹, Ana Paula Sakamoto¹⁰, Ekaterina Alexeeva¹¹, Jordi Anton¹², Maria Katsicas¹³, Vanessa Smith¹⁴, Tadej Avcin¹⁵, Edoardo Marrani¹⁶, Mikhail Kostik¹⁷, Thomas Lehman¹⁸, Walter Alberto Sifuentes-Giraldo¹⁹, Simone Appenzeller²⁰, Mahesh Janarthanan²¹, Monika Moll²², Dana Nemcova²³, Dieneke Schonenberg-Meinema²⁴, Cristina Battagliotti²⁵, Lillemor Berntson²⁶, Blanca Bica²⁷, Jürgen Brunner²⁸, Patricia Costa Reis²⁹, Despina Eleftheriou³⁰, Liora Harel³¹, Gerd Horneff³², Tilmann Kallinich³³, Dragana Lazarevic³⁴, Kirsten Minden³⁵, Susan Nielsen³⁶, Farzana Nuruzzaman³⁷, Anjali Patwardhan³⁸, Yosef Uziel³⁹ and Nicola Helmus⁴⁰, ¹Head of the Hamburg Centre for Pediatric and Adolescence Rheumatology, Budapest, Hungary, ²Kinderklinik Heidberg Pulmologie, Hamburg, Germany, ³University of Pittsburgh, Pittsburgh, PA, ⁴Hospital Garcia de Orta, Almada, Portugal, ⁵PRINTO, Istituto Giannina Gaslini, Genova, Italy, ⁶Cerrahpaşa Tıp Fakültesi, Istanbul, Turkey, ⁷Riga Stradins University, Riga, Latvia, ⁸Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, ⁹Federal University of São Paulo, São Paulo, Brazil, ¹⁰UNIFESP, Sao Paulo, Brazil, ¹¹Scientific Center of Children’s Health of RAMS, Moscow, Russia, ¹²Sant Joan de Déu Hospital, Madrid, Spain, ¹³Hospital de Pediatria, Buenos Aires, Argentina, ¹⁴Department of Rheumatology, Ghent University Hospital, Department of Internal Medicine, VIB Inflammation Research Centre Ghent University, Ghent, Belgium, ¹⁵Ljubljana University Medical Centre, Ljubljana, Slovenia, ¹⁶University of Florence, Firenze, Italy, ¹⁷Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ¹⁸Hospital for Special Surgery, New York, ¹⁹Hospital Universitario Ramon y Cajal, Madrid, Spain, ²⁰Unicamp, Campinas, Brazil, ²¹Sri Ramachandra University, Chennai, India, ²²University of Tübingen, Tübingen, Germany, ²³General University Hospital, Prague, Czech Republic, ²⁴Amsterdam UMC, Amsterdam, Netherlands, ²⁵Children's Hospital Dr. Orlando Alassia, Santa Fee, Argentina, ²⁶Uppsala University Hospital, Uppsala, Sweden, ²⁷Hospital Universitário Clementino Fraga Filho (HUCFF/UFRJ), Rio de Janeiro, Brazil, ²⁸Tirol Kliniken, Innsbruck, Innsbruck, Austria, ²⁹Hospital de Santa Maria, Lisbon, Portugal, ³⁰Great Ormond Street Hospital, London, United Kingdom, ³¹Clalit Health Services, Nettnja, Israel, ³²Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, ³³Charite, Berlin, Germany, ³⁴KC Niš, Nis, Serbia, ³⁵Deutsches Rheuma-Forschungszentrum Berlin, Berlin, Germany, ³⁶Rigshospitalet, Copenhagen, Denmark, ³⁷Stony Brook Children's Hospital, Stony Brook, NY, ³⁸University of Missouri, Columbia, ³⁹Meir Medical Center, Kfar Saba, Israel, ⁴⁰Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany

Meeting: ACR Convergence 2020

Keywords: interstitial lung disease, Pediatric rheumatology, Scleroderma, Systemic

Session Information

Date: Sunday, November 8, 2020

Title: Pediatric Rheumatology – Clinical Poster II: Systemic JIA, Autoinflammatory, & Scleroderma

Session Type: Poster Session C

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

Background/Purpose:

Juvenile systemic sclerosis (jSSc) has a prevalence in around 3 in a million children. Pulmonary involvement occurs in approximately 40 % in the international juvenile systemic scleroderma cohort (JSScC). 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 interstitial lung disease (ILD) in children.

Methods: JSScC 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.

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 cohort, supporting the use of HRCT for detection of ILD. The cut off utilized, of less than 80% of predicted FVC or DLCO could be too low to exclude beginning ILD.

Supported by the “Joachim Herz Stiftung”

Disclosure: I. Foeldvari, Sanofi, 5, Chugai, 5, Amgen, 5, GSK, 5, Lilly, 5, BMS, 5, Abbvie, 5, Novartis, 5, gilead, 5; B. Hinrichs, None; K. Torok, None; M. Santos, None; O. Kasapcopur, None; A. Adrovic, None; V. Stanevicha, None; F. Sztajnbok, None; M. Terreri, None; A. Sakamoto, None; E. Alexeeva, Novartis, 2, 5, 8, Roche, 2, 5, 8, Pfizer, 2, 5, 8, AbbVie, 2, 5, 8; J. Anton, Sobi, 1, 2, 3, Novartis, 1, 2, 3, GSK, 1, 2, 3, Pfizer, 1, 2, BMS, 1, Chemocentryx,, 1, Lilly, 1, Novimmune, 1, Sanofi, 1, Roche, 1; M. Katsicas, None; V. Smith, Boehringer Ingelheim, 2, 5, 8, Janssen, 2, 5, 8; T. Avcin, AbbVie, 5, 8, Alexion, 5, Octapharma, 5, 8, Takeda, 5, 8; E. Marrani, 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-Meinema, 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, Pfizer, 5, 8, AbbVie, 5, 8, Novartis, 5, 8, Sanofi, 5, 8; T. Kallinich, None; D. Lazarevic, None; K. Minden, Sanofi, 1, gsk, 1, Roche, 1, Abbvie, 1, Biermann, 8, Medac, 8; S. Nielsen, None; F. Nuruzzaman, None; A. Patwardhan, None; Y. Uziel, Pfizer, 8; 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, Sakamoto A, Alexeeva E, Anton J, Katsicas M, Smith V, Avcin T, Marrani E, Kostik M, Lehman T, Sifuentes-Giraldo W, Appenzeller S, Janarthanan M, Moll M, Nemcova D, Schonenberg-Meinema 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. 2020; 72 (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-2/. Accessed .

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