Systemic Juvenile Idiopathic Arthritis Associated Lung Disease in Europe

Claudia Bracaglia¹, Francesca Minoia², Christoph Kessel³, Sebastiaan Vastert⁴, Manuela Pardeo¹, Alessia Arduini¹, Sarka Fingerhutova⁵, Irina Nikishina⁶, Ozge Basaran⁷, Nural Kiper⁸, Mikhail Kostik⁹, Mia Glerup¹⁰, Roberta Caorsi¹¹, AnnaCarin Horne¹², Giovanni Filocamo², Helmut Wittkowski³, Marija Jelusic¹³, Jordi Anton¹⁴, Samira Khaldi-Plassart¹⁵, Alexandre Belot¹⁵, Gerd Horneff¹⁶, Seraina Palmer Sarrott¹⁷, Elvira Cannizzaro Schneider¹⁸, Lampros Fotis¹⁹, Pavla Dolezalova⁵, Angelo Ravelli²⁰, Seza Ozen⁷ and Fabrizio De Benedetti¹, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy, ²Fondazione IRCCS Ca' Grande Ospedale Maggiore Policlinico, Milano, Italy, ³Department of Pediatric Rheumatology & Immunology, WWU Medical Center (UKM), Muenster, Germany, ⁴Wilhelmina Children’s Hospital, Department of Pediatric Immunology and Rheumatology, Utrecht, The Netherlands, Utrecht, Netherlands, ⁵Centre for Paediatric Rheumatology and Autoinflammatory Diseases, Department of Paediatrics and Inherited Metabolic Disorders, 1st Faculty of Medicine, Charles University and General University Hospital in Prague, Prague, Czech Republic, ⁶V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia, ⁷Department of Pediatrics, Division of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, ⁸Department of Pediatrics, Division of Pediatric Pulmonology, Hacettepe University, Ankara, Turkey, ⁹Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, ¹⁰Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark, ¹¹Department of Pediatrics and Rheumatology, IRRCS Istituto G. Gaslini, Genova, Italy, ¹²Department of pediatric rheumathology Karolinska University Hospital and Department of pediatrics, Karolinska Institute, Stockholm, Sweden, ¹³Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre, Zagreb, Croatia, ¹⁴Pediatric Rheumatology, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain, ¹⁵Pediatric Nephrology, Rheumatology, Dermatology Unit, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Lyon, France, ¹⁶Pediatrics, Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, ¹⁷Paediatric Rheumatology University Children’s Hospital Zurich, Zurich, Zurich, Switzerland, ¹⁸Paediatric Rheumatology University Children’s Hospital Zurich, Zurich, Switzerland, ¹⁹Pediatric Rheumatology Division, 3rd Department of Pediatrics, National and Kapodistrian University of Athens,‘’ATTIKON’’ General University Hospital, Athens, Greece, ²⁰IRRCS Istituto Giannina Gaslini and Università degli Studi di Genova, Genova, Italy

Meeting: 2023 Pediatric Rheumatology Symposium

Keywords: interstitial lung disease, Juvenile idiopathic arthritis, macrophage activation syndrome

Session Information

Date: Friday, March 31, 2023

Title: Posters: Clinical and Therapeutic II

Session Type: Poster Session B

Session Time: 5:00PM-6:00PM

Background/Purpose: Chronic parenchymal lung disease (LD) is a new emerging severe life-threatening complication of sJIA. The number of sJIA patients with LD is apparently increasing and interestingly they are reported more frequently in North America. Data regarding frequency and features of sJIA-LD in Europe are not available. The aim of this study was to evaluate the burden of sJIA associated LD in Europe.

Methods: Patients with diagnosis of sJIA with LD, including pulmonary alveolar proteinosis (PAP), interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH), followed in European paediatric rheumatology centres were identified through a survey sent to the members of the MAS/SJIA Working Party.

Results: Data from 49 JIA-LD patients, diagnosed in 17 European paediatric rheumatology centres between 2007 and 2022, were collected. 48 patients were Caucasian and 1 was African-American; 31 were female. The median age at sJIA onset was 7 years and LD occurred after a median time of 3 years. 27 patients had a chronic persistent sJIA course, 21 had a polycyclic course and only 1 patient had a monocyclic course; 38 patients (77%) had active sJIA at time of LD diagnosis. During the disease course, 41 (84%) patients developed MAS, 18 (44%) of whom had MAS at sJIA onset and 24 (58%) had full-blown MAS at time of LD diagnosis; 31 (76%) patients had 1 MAS episode. 42 (86 %) patients were treated with at least one IL-1 or IL-6 inhibitor before LD diagnosis: 33 with anakinra, 26 with canakinumab, and 23 with tocilizumab; 22 (45%) patients experienced drug adverse reaction to a cytokine inhibitor: 14 to tocilizumab and 6 to anakinra, and 1 patient to cyclosporine. 39 (80%) patients developed ILD, 6 (12%) PAP and 4 (8%) PAH. 22 (45%) patients presented acute digital clubbing; 18 (37%) patients developed hypoxia and 9 (18%) developed pulmonary hypertension. A chest CT scan was performed in all patients with evidence of septal thickening, peri-bronchovascular thickening and ground glass opacities in the majority of patients (39, 25 and 28 patients respectively). In 22 patients a bronchoalveolar lavage was performed and 15 underwent a lung biopsy. The histopathological pattern was alveolar proteinosis in 5 patients, endogenous lipoid pneumonia in 5, vasculitis in 1 and fibrosis in 1. 45 out of 49 patients were treated with glucocorticoids (GCs) at time of LD diagnosis, and 39 received IL-1 and/or IL-6 inhibitor after the diagnosis (25 anakinra, 20 canakinumab, 16 tocilizumab). Around half of the patients (23, 47%) required ICU admission and 9 (18%) died.

Conclusion: Lung involvement is an emerging life-threatening complication of sJIA, patients are also reported in Europe. Prompt recognition is crucial and new therapeutic strategies are needed to reduce the risk and improve the outcome of this complication.

This abstract has been submitted on behalf of MAS/sJIA Working Party of PReS.

Disclosures: C. Bracaglia: Sobi, 2, 6; F. Minoia: Sobi, 2; C. Kessel: Novartis, 2, 5, Sobi, 6; S. Vastert: Novartis, 6, SOBI, 5, 6; M. Pardeo: SOBI, 2, 6; A. Arduini: None; S. Fingerhutova: None; I. Nikishina: Ipsen, 6, Merck/MSD, 6, Novartis, 6, Pfizer, 6, Roche, 6, R-Pharm, 6, Sobi, 6; O. Basaran: None; N. Kiper: None; M. Kostik: None; M. Glerup: None; R. Caorsi: Novartis, 2, Sobi, 2; A. Horne: None; G. Filocamo: Sobi, 2; H. Wittkowski: None; M. Jelusic: None; J. Anton: AbbVie/Abbott, 5, Amgen, 5, Bristol-Myers Squibb(BMS), 5, Eli Lilly, 5, GlaxoSmithKlein(GSK), 2, 5, 6, Novartis, 2, 5, 6, Novimmune, 2, 5, 6, Pfizer, 2, 5, 6, Roche, 5, Sanofi, 5, Sobi, 2, 5, 6; S. Khaldi-Plassart: None; A. Belot: Novartis, 2, Pfizer, 2, Roche, 2, Sobi, 2; G. Horneff: AbbVie/Abbott, 6, Chugai, 6, Eli Lilly, 6, Merck/MSD, 5, Novartis, 5, 6, Pfizer, 6, Roche, 5, Sanofi, 6; S. Palmer Sarrott: None; E. Cannizzaro Schneider: None; L. Fotis: None; P. Dolezalova: None; A. Ravelli: AbbVie/Abbott, 6, Alexion, 6, Angelini, 6, Bristol-Myers Squibb(BMS), 6, Novartis, 6, Pfizer, 6, Reckitt Benckiser, 6, Roche, 6, Sobi, 6; S. Ozen: Novartis, 2, 6, Sobi, 2, 6; F. De Benedetti: AbbVie/Abbott, 2, Novartis, 2, Novimmune, 2, Pfizer, 2, Roche, 2, Sobi, 2.

To cite this abstract in AMA style:

Bracaglia C, Minoia F, Kessel C, Vastert S, Pardeo M, Arduini A, Fingerhutova S, Nikishina I, Basaran O, Kiper N, Kostik M, Glerup M, Caorsi R, Horne A, Filocamo G, Wittkowski H, Jelusic M, Anton J, Khaldi-Plassart S, Belot A, Horneff G, Palmer Sarrott S, Cannizzaro Schneider E, Fotis L, Dolezalova P, Ravelli A, Ozen S, De Benedetti F. Systemic Juvenile Idiopathic Arthritis Associated Lung Disease in Europe [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 4). https://acrabstracts.org/abstract/systemic-juvenile-idiopathic-arthritis-associated-lung-disease-in-europe-2/. Accessed .

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