Systemic Juvenile Idiopathic Arthritis Associated Lung Disease in Europe

Claudia Bracaglia¹, Francesca Minoia², Christoph Kessel³, Sebastiaan Vastert⁴, Manuela Pardeo¹, Alessia Arduini¹, Ozge Basaran⁵, Nural kiper⁶, Mikhail Kostik⁷, Mia Glerup⁸, Sarka Fingerhutova⁹, Roberta Caorsi¹⁰, AnnaCarin Horne¹¹, Giovanni Filocamo¹², Helmut Wittkowski³, Marija Jelusic¹³, Jordi Anton¹⁴, Samira Khaldi-Plassart¹⁵, Alexandre Belot¹⁶, Gerd Horneff¹⁷, Seraina Palmer Sarott¹⁸, Elvira cannizzaro Schneider¹⁸, Pavla Dolezalova⁹, Angelo Ravelli¹⁹, Seza Ozen²⁰ and Fabrizio De Benedetti¹, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, ²Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy, ³Department of Pediatric Rheumatology & Immunology, WWU Medical Center (UKM), Münster, Germany, ⁴Pediatric Rheumatology & Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ⁵Department of Pediatrics, Division of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, ⁶Department of Pediatrics, Division of Pediatric Pulmonology, Hacettepe University, Ankara, Turkey, ⁷Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ⁸Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark, ⁹Centre 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, ¹⁰Department of pediatrics and Rheumatology, IRRCS Istituto G. Gaslini, Genova, Italy, ¹¹Department of pediatric rheumathology Karolinska University Hospital and Department of pediatrics, Karolinska Institute, Stockholm, Sweden, ¹²UOC Pediatria a Media Intensità di Cure, Clinica de Marchi, Milano, Italy, ¹³Department of Pediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, 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, ¹⁶Hospices Civils de Lyon, Collonges au mont d'or, France, ¹⁷Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ¹⁸Paediatric Rheumatology University Children’s Hospital Zurich, Zürich, Switzerland, ¹⁹Department of Neurosciences, Rehabilitation, Ophthalmology, Genetic and Maternal Infantile Sciences (DINOGMI) University of Genoa, Italy,Scientific Direction, IRCCS Istituto Giannina Gaslini, Genova, Italy, ²⁰Hacettepe University Faculty of Medicine, Ankara, Turkey

Meeting: ACR Convergence 2022

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

Session Information

Date: Sunday, November 13, 2022

Title: Pediatric Rheumatology – Clinical Poster I: JIA

Session Type: Poster Session B

Session Time: 9:00AM-10:30AM

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 if this study was to evaluate the burden of sJIA-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 34 sJIA-LD patients, diagnosed in 15 European paediatric rheumatology centres between 2007 and 2022, were collected. 33 patients were Caucasian and 1 was African-American; 21 were female. The median age at sJIA onset was 6 years and LD occurred after a median time of 2 years. 19 patients had a chronic persistent sJIA course, 14 had a polycyclic course and only 1 patient had a monocyclic course; 29 (85%) had active sJIA at time of LD diagnosis. During the disease course, 28 (82%) patients developed MAS, 12 (35%) of whom had MAS at sJIA onset and 19 (56%) had full-blown MAS at time of LD diagnosis; 23 (68%) patients had >1 MAS episode. 28 (82 %) patients were treated with at least one IL-1 or IL-6 inhibitor before LD diagnosis: 15 with canakinumab, 24 with anakinra and 13 with tocilizumab; 13 (38%) patients experienced drug adverse reaction to a cytokine inhibitor: 9 to tocilizumab and 4 to anakinra. 24 (70%) patients developed ILD, 6 (18%) PAP and 4 (12%) PAH. 15 (44%) patients presented acute digital clubbing; 16 (47%) patients developed hypoxia and 9 (26%) 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 (26, 18 and 18 respectively). In 17 patients a bronchoalveolar lavage was performed and 12 underwent a lung biopsy. The histopathological pattern was alveolar proteinosis in 5 patients, endogenous lipoid pneumonia in 3, vasculitis in 1 and fibrosis in 1. Half of the patients (17) required ICU admission and 6 (18%) died. All the patients were treated with glucocorticoids (GCs) at time of diagnosis, and 26 received IL-1 or IL-6 inhibitor after the diagnosis (13 canakinumab, 20 anakinra, 14 tocilizumab).

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

Disclosures: C. Bracaglia, SOBI, Novartis; F. Minoia, SOBI; C. Kessel, SOBI, Novartis, Novartis; S. Vastert, None; M. Pardeo, None; A. Arduini, None; O. Basaran, None; N. kiper, None; M. Kostik, None; M. Glerup, None; S. Fingerhutova, None; R. Caorsi, Novartis, SOBI; A. Horne, None; G. Filocamo, None; H. Wittkowski, None; M. Jelusic, None; J. Anton, for Sobi, Novimmune, Novartis, Abbvie, Pfizer, GSK, Roche, Amgen, Lilly, BMS, Sanofi, Sobi, Novimmune, Novartis, Pfizer, GSK, Sobi, Novimmune, Novartis, GSK, Pfizer.; S. Khaldi-Plassart, None; A. Belot, SOBI, Novartis, Roche, Pfizer; G. Horneff, Roche, Pfizer, Novartis, Merck/MSD, Eli Lilly, AbbVie/Abbott; S. Palmer Sarott, None; E. cannizzaro Schneider, None; P. Dolezalova, None; A. Ravelli, None; S. Ozen, None; F. De Benedetti, Sobi, Novimmune, Pfizer, Novartis, Roche, Abbvie.

To cite this abstract in AMA style:

Bracaglia C, Minoia F, Kessel C, Vastert S, Pardeo M, Arduini A, Basaran O, kiper N, Kostik M, Glerup M, Fingerhutova S, Caorsi R, Horne A, Filocamo G, Wittkowski H, Jelusic M, Anton J, Khaldi-Plassart S, Belot A, Horneff G, Palmer Sarott S, cannizzaro Schneider E, Dolezalova P, Ravelli A, Ozen S, De Benedetti F. Systemic Juvenile Idiopathic Arthritis Associated Lung Disease in Europe [abstract]. Arthritis Rheumatol. 2022; 74 (suppl 9). https://acrabstracts.org/abstract/systemic-juvenile-idiopathic-arthritis-associated-lung-disease-in-europe/. Accessed .

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