Still’s Disease Associated Lung Disease: Update From the European Registry

Claudia Bracaglia¹, Francesca Minoia², Christoph Kessel³, Sebastiaan Vastert⁴, Manuela Pardeo⁵, Alessia Arduini⁵, Sarka Horackova Fingerhutova⁶, Irina Nikishina⁷, Ozge Basaran⁸, Nural Kiper⁸, Konstantin Belozerov⁹, Mikhail Kostik⁹, Sezgin Sahin¹⁰, Mia Glerup¹¹, Roberta Caorsi¹², AnnaCarin Horne¹³, Giovanni Filocamo¹⁴, Helmut Wittkowski¹⁵, Marija Jelusic¹⁶, Jordi Anton¹⁷, Samira Khaldi-Plassart¹⁸, Alexandre Belot¹⁸, Gerd Horneff¹⁹, Seraina Palmer Sarott²⁰, Elvira Cannizzaro Schneider²⁰, Lampros Fotis²¹, Serena Pastore²², Aurelia Calin²³, Isabelle Kone-Paut²⁴, Ozgur Kasapcopur¹⁰, Pavla Dolezalova⁶, Seza zen²⁵, Angelo Ravelli²⁶ and Fabrizio De Benedetti²⁷, ¹Division of Rheumatology Ospedale Pediatrico Bambino Gesu' IRCCS, Roma, Italy, Rome, Rome, Italy, ²Fondazione IRCCS Ca' Grande Ospedale Maggiore Policlinico, Milan, Italy,, Milano, Italy, ³Department of Pediatric Rheumatology & Immunology, WWU Medical Center (UKM), Münster, Germany, Munster, Germany, ⁴University Medical Center Utrecht, Utrecht, Netherlands, ⁵Division of Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy, Rome, Lazio, Italy, ⁶Centre 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, Prague, Czech Republic, ⁷V.A. Nasonova Research Institute of Rheumatology, Moscow, Russian Federation, Moscow, Russia, ⁸Department of Pediatrics, Hacettepe University, Ankara, Türkiye, Ankara, Turkey, ⁹Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russian Federation, Saint-Petersburg, Russia, ¹⁰Istanbul University-Cerrahpasa, Department of Pediatric Rheumatology, Istanbul, Türkiye, Istanbul, Turkey, ¹¹Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark, Aarhus, Denmark, ¹²Department of Pediatrics and Rheumatology, IRRCS Istituto G. Gaslini, Genova, Italy, Genova, Italy, ¹³Department of pediatric rheumathology, Karolinska University Hospital and Department of pediatrics, Karolinska Institute, Stockholm, Sweden, Stockholm, Sweden, ¹⁴Fondazione IRCCS Ca' Grande Ospedale Maggiore Policlinico, Milan, Italy, Milano, Italy, ¹⁵Department of Pediatric Rheumatology & Immunology, WWU Medical Center (UKM), Münster, Germany, Munster, Italy, ¹⁶Department of Pediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia, Zagreb, Croatia, ¹⁷Pediatric Rheumatology, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain, Barcelona, Spain, ¹⁸Pediatric Nephrology, Rheumatology, Dermatology Unit, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Lyon, France, Lyon, France, ¹⁹University Hospital of Cologne, Sankt Augustin, Germany, ²⁰Department of Pediatric Rheumatology, University Children’s Hospital Zurich, Zurich, Switzerland, Zurich, Switzerland, ²¹Pediatric Rheumatology Division, ³rd Department of Pediatrics, National and Kapodistrian University of Athens,‘’ATTIKON’’ General University Hospital, Athens, Greece, Athens, Greece, ²²IRCCS Burlo Garofolo, Trieste, Trieste, Italy, ²³Marie Curie Children's Emergency Hospital, Bucharest, Romania, Bucharest, Romania, ²⁴Pediatric rheumatology department, CEREMAIA Bicêtre hospital, APHP, Le Kremlin Bicêtre University of Paris Saclay, Paris, France, Paris, France, ²⁵Hacettepe University Medical Faculty, Ankara, Turkey, ²⁶IRCCS Istituto Giannina Gaslini, Genoa, Italy, Genoa, Genoa, Italy, ²⁷Bambino Gesu Children's Hospital, Rome, Rome, Italy

Meeting: 2026 Pediatric Rheumatology Symposium

Session Information

Date: Friday, March 20, 2026

Title: Plenary Abstract Session II

Session Time: 2:30PM-2:45PM

Background/Purpose: Lung disease (LD) is an emerging severe life-threatening complication of Still’s Disease (SD) characterized by peculiar features. Patients with SD complicated by LD are increasing and available data are essentially provided by North American series. Frequency and features of SD-associated LD (SD-LD) in Europe have never been reported.

Methods: Patients with diagnosis of SD-LD, followed at European paediatric rheumatology centres were identified through a survey sent to the members of the MAS/sJIA Working Party of PReS.

Results: Data from 64 patients with SD-LD, diagnosed at 21 European paediatric rheumatology centres between 2007 and 2025, were collected. 62 patients were White-Caucasian and 2 African American; 39 were female. The median age at SD onset was 3.5 years and LD occurred after a median time of 1.9 years. 3 patients (5%) had trisomy 21. 86% (55) of the patients had at least one episode of MAS, 26 (47%) of whom had MAS at SD onset and 33 (60%) at time of LD diagnosis; 39 (71%) patients had >1 MAS episode. Clinical and lung features were overall comparable to those reported in previous described cohort (1).
IL-18 levels, measured in 52 (81%) patients, were markedly elevated at time of SD onset and of LD diagnosis (median of 56.686 and 137.500 pg/ml respectively). Eosinophils count was measured in 56 (87%) patients, and it was >1.000 cell/mmc in 10 (21%) patients at time of SD onset and in 15 (27%) at time of LD diagnosis. HLA-DRB1 genotyping was performed in half of the patients (32/64). The HLA-DRB1*15 allele was found positive in 21/32 (65%) patients, while the HLA-DRB1*11 was positive in 15/32 (47%) patients. Twenty-seven (42%) patients experienced adverse drug reaction to a cytokine inhibitor: 13 to tocilizumab, 9 to anakinra and 5 to both.
Twelve patients (19%) did not receive either IL-1 or IL-6 inhibitors (IL-1/IL-6i) before LD diagnosis. The age at SD onset was comparable to that of patients who received IL-1/IL-6i prior to LD onset. Interestingly, disease duration at LD onset was significantly shorter in patients who had not received IL-1/IL-6i (p=0.02), raising the hypothesis that treatment with IL-1/IL-6i may control better SD and therefore, potentially delay LD onset.
History of MAS and outcome of SD (ICU admission and death) were comparable in the two groups.
After LD diagnosis, 55 (86%) patients received intravenous or oral glucocorticoids and additional treatments that were very heterogeneous across the entire cohort. Eight patients underwent hematopoietic stem cell transplantation.
At last follow-up, LD worsened in 14 patients and 21 developed complications: 12 hypoxia, 8 O2 requirement and 8 pulmonary hypertension. 29 patients (45%) required ICU admission and 11 (17%) died.

Conclusion: SD-LD is seen in European patients. Clinical and lung features are comparable to those described in North American cohorts. Around 1 out of 5 of our patients did not receive IL-1/IL-6i prior to LD onset and had shorter disease duration at LD onset. These observations argue against a direct role of IL-1/IL-6i in the development of LD.
References.
< !1. Huang Y et al. Arthritis Care Res. 2024 Mar;76(3):328-339.

Disclosures: C. Bracaglia: GlaxoSmithKlein(GSK), 6, Novartis, 2, Sobi, 2; F. Minoia: Novartis, 6, Sobi, 2; C. Kessel: Novartis, 2, 5, Sobi, 6; S. Vastert: Novartis, 2, Sobi, 2; M. Pardeo: Novartis, 2, Sobi, 2; A. Arduini: None; S. Horackova 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; K. Belozerov: None; M. Kostik: None; S. Sahin: 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, 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, 6, Chugai, 6, Eli Lilly, 6, Merck/MSD, 5, Novartis, 5, 6, Pfizer, 6, Roche, 5, Sanofi, 6; S. Palmer Sarott: None; E. Cannizzaro Schneider: None; L. Fotis: None; S. Pastore: None; A. Calin: None; I. Kone-Paut: None; O. Kasapcopur: None; P. Dolezalova: None; S. Özen: Novartis, 2, 6, Sobi, 2, 6; A. Ravelli: Abbvie, 2, Galapagos, 2, Novartis, 2, Pfizer, 2, Roche, 2, Sobi, 2; F. De Benedetti: Abbvie, 2, 5, Apollo, 2, 5, Elixiron, 2, 5, Kiniksa, 2, 5, Novartis, 2, 5, Sanofi, 2, 5, Sobi, 2, 5.

To cite this abstract in AMA style:

Bracaglia C, Minoia F, Kessel C, Vastert S, Pardeo M, Arduini A, Horackova Fingerhutova S, Nikishina I, Basaran O, Kiper N, Belozerov K, Kostik M, Sahin S, Glerup M, 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, Fotis L, Pastore S, Calin A, Kone-Paut I, Kasapcopur O, Dolezalova P, Özen S, Ravelli A, De Benedetti F. Still’s Disease Associated Lung Disease: Update From the European Registry [abstract]. Arthritis Rheumatol. 2026; 78 (suppl 3). https://acrabstracts.org/abstract/stills-disease-associated-lung-disease-update-from-the-european-registry/. Accessed .

« Back to 2026 Pediatric Rheumatology Symposium

ACR Meeting Abstracts - https://acrabstracts.org/abstract/stills-disease-associated-lung-disease-update-from-the-european-registry/