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
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.
