The Systemic Score May Identify Life‐Threatening Evolution in Still Disease: Data from the GIRRCS AOSD‐Study Group and the AIDA Network Still Disease Registry-Reference-Cited by-同舟云学术

The Systemic Score May Identify Life‐Threatening Evolution in Still Disease: Data from the GIRRCS AOSD‐Study Group and the AIDA Network Still Disease Registry

Published:2024-05-15 Issue:7 Volume:76 Page:1141-1152
ISSN:2326-5191
Container-title:Arthritis & Rheumatology
language:en
Short-container-title:Arthritis & Rheumatology

Author:

Ruscitti Piero¹^ORCID,Masedu Francesco¹,Vitale Antonio²³,Caggiano Valeria²³,Di Cola Ilenia¹,Cipriani Paola¹,Valenti Marco¹,Mayrink Giardini Henrique A⁴^ORCID,de Brito Antonelli Isabele Parente⁴,Dagostin Marilia Ambiel⁴,Lopalco Giuseppe⁵,Iannone Florenzo⁵^ORCID,Maria Morrone⁵,Almaghlouth Ibrahim A⁶⁷,Asfina Kazi Nur⁷,Ali Hebatallah Hamed⁷,Ciccia Francesco⁸^ORCID,Iacono Daniela⁸,Pantano Ilenia⁸,Mauro Daniele⁸,Sfikakis Petros P⁹^ORCID,Tektonidou Maria⁹^ORCID,Laskari Katerina⁹^ORCID,Berardicurti Onorina¹⁰¹¹,Dagna Lorenzo¹²¹³,Tomelleri Alessandro¹²¹³^ORCID,Tufan Abdurrahman¹⁴,Can Kardas Rıza¹⁴^ORCID,Hinojosa‐Azaola Andrea¹⁵,Martín‐Nares Eduardo¹⁵^ORCID,Kawakami‐Campos Perla Ayumi¹⁶,Ragab Gaafar¹⁷¹⁸,Hegazy Mohamed Tharwat¹⁷¹⁸,Direskeneli Haner¹⁹,Alibaz‐Oner Fatma¹⁹^ORCID,Fotis Lampros²⁰,Sfriso Paolo²¹,Govoni Marcello²²,La Torre Francesco²³,Cristina Maggio Maria²⁴,Montecucco Carlomaurizio²⁵²⁶,De Stefano Ludovico²⁵²⁶,Bugatti Serena²⁵²⁶,Rossi Silvia²⁶,Makowska Joanna²⁷,Del Giudice Emanuela²⁸,Emmi Giacomo²⁹³⁰,Bartoloni Elena³¹^ORCID,Hernández‐Rodríguez José³²,Conti Giovanni³³,Nunzia Olivieri Alma³⁴,Lo Gullo Alberto³⁵,Simonini Gabriele³⁶,Viapiana Ombretta³⁷,Wiesik‐Szewczyk Ewa³⁸,Erten Sukran³⁹,Carubbi Francesco⁴⁰,De Paulis Amato⁴¹,Maier Armin⁴²,Tharwat Samar⁴³⁴⁴,Costi Stefania⁴⁵,Iagnocco Annamaria⁴⁶,Sebastiani Gian Domenico⁴⁷,Gidaro Antonio⁴⁸,Brucato Antonio Luca⁴⁹,Karamanakos Anastasios⁵⁰,Akkoç Nurullah⁵¹,Caso Francesco⁵²,Costa Luisa⁵²^ORCID,Prete Marcella⁵³,Perosa Federico⁵³,Atzeni Fabiola⁵⁴^ORCID,Guggino Giuliana⁵⁵^ORCID,Fabiani Claudia²³,Frediani Bruno²³,Giacomelli Roberto¹⁰¹¹,Cantarini Luca²³

Affiliation:

1. Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences University of L'Aquila L'Aquila Italy

2. Department of Medical Sciences Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena Siena Italy

3. Azienda Ospedaliero‐Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena Italy

4. Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo São Paulo Brazil

5. Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe‐J) Policlinic Hospital University of Bari Bari Italy

6. Rheumatology Unit, Department of Medicine, College of Medicine King Saud University Riyadh Saudi Arabia

7. College of Medicine Research Center, College of Medicine, King Saud University Riyadh Saudi Arabia

8. Department of Precision Medicine University of Campania “Luigi Vanvitelli” Naples Italy

9. Joint Academic Rheumatology Program, Medical School National and Kapodistrian University of Athens Athens Greece

10. Clinical and Research Section of Rheumatology and Clinical Immunology Fondazione Policlinico Campus Bio‐Medico, Via Álvaro del Portillo 200 Rome Italy

11. Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine University of Rome “Campus Biomedico” School of Medicine Rome Italy

12. Division of Immunology, Transplants and Infectious Diseases Università Vita‐Salute San Raffaele Milan Italy

13. Unit of Immunology, Rheumatology, Allergy and Rare Diseases IRCCS Ospedale San Raffaele Milan Italy

14. Department of Internal Medicine, Division of Rheumatology Gazi University Hospital Ankara Turkey

15. Department of Immunology and Rheumatology Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City Mexico

16. Department of Ophthalmology Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán Mexico City Mexico

17. Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine Cairo University Egypt

18. Faculty of Medicine Newgiza University (NGU) Egypt

19. Department of Internal Medicine, Division of Rheumatology Marmara University, Faculty of Medicine Istanbul Turkey

20. Department of Pediatrics, Attikon General Hospital National and Kapodistrian University of Athens Greece

21. Rheumatology Unit, Department of Medicine University of Padua Padua Italy

22. Rheumatology Unit, Department of Medical Sciences, Azienda Ospedaliero‐Universitaria S. Anna‐Ferrara University of Ferrara Ferrara Italy

23. Department of Pediatrics, Pediatric Rheumatology Center, Giovanni XXIII Pediatric Hospital University of Bari Bari Italy

24. University Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE) “G.D'Alessandro” University of Palermo Palermo Italy

25. Department of Internal Medicine and Therapeutics University of Pavia Italy

26. Division of Rheumatology Fondazione IRCCS Policlinico San Matteo Pavia Italy

27. Department of Rheumatology Medical University of Lodz, Zeromskiego 113, Lodz Poland

28. Pediatric and Neonatology Unit, Department of Maternal Infantile and Urological Sciences Sapienza University of Rome Latina Italy

29. Department of Experimental and Clinical Medicine University of Florence Florence Italy

30. Centre for Inflammatory Diseases, Department of Medicine, Monash Medical Centre Monash University Clayton VIC Australia

31. Rheumatology Unit, Department of Medicine and Surgery University of Perugia Perugia Italy

32. Department of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic of Barcelona [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] University of Barcelona Barcelona Spain

33. Pediatric Nephrology and Rheumatology Unit, Azienda Ospedaliera Universitaria (AOU), “G. Martino” Messina Italy

34. Department of Woman, Child and of General and Specialized Surgery University of Campania “Luigi Vanvitelli” Naples Italy

35. UOSD Reumatologia, ARNAS Garibaldi Catania Italy

36. NEUROFARBA Department, Rheumatology Unit, MeyerChildren's Hospital IRCCS University of Florence Florence Italy

37. Rheumatology Unit, Department of Medicine University and Azienda Ospedaliera Universitaria Integrata of Verona Italy

38. Department of Internal Medicine, Pneumonology, Allergology and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense Military Institute of Medicine, National Research Institute Warsaw Poland

39. Department of Rheumatology, Faculty of Medicine Ankara City Hospital Ankara Yıldırım Beyazıt University Ankara Turkey

40. Department of Life, Health & Environmental Sciences and Internal Medicine and Nephrology Unit, Department of Medicine University of L'Aquila and ASL Avezzano‐Sulmona‐L'Aquila, San Salvatore Hospital L'Aquila Italy

41. Department of Translational Medical Sciences, Section of Clinical Immunology University of Naples Federico II Naples Italy

42. Rheumatology Unit, Department of Medicine, Central Hospital of Bolzano Bolzano Italy

43. Rheumatology and Immunology Unit, Internal Medicine Department Mansoura University Mansoura Egypt

44. Department of Internal Medicine, Faculty of Medicine Horus University New Damietta Egypt

45. Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases University of Milan Milan Italy

46. Academic Rheumatology Centre, Ospedale Mauriziano ‐ Dipartimento Scienze Cliniche e Biologiche Università degli Studi di Torino Turin Italy

47. UOC di Reumatologia Azienda Ospedaliera San Camillo Forlanini Roma Italy

48. Department of Biomedical and Clinical Sciences Luigi Sacco, Luigi Sacco Hospital University of Milan Milan Italy

49. Pediatric Unit, Fatebenefratelli Hospital Milan Italy

50. Joint Academic Rheumatology Program, First Department of Propaedeutic and Internal Medicine National and Kapodistrian University of Athens Athens Greece

51. Division of Rheumatology, Department of Internal Medicine, School of Medicine Manisa Celal Bayar University Manisa Turkey

52. Rheumatology Unit, Department of Clinical Medicine and Surgery University of Naples Federico II Naples Italy

53. Rheumatic and Systemic Autoimmune Diseases Unit, Department of Interdisciplinary Medicine (DIM) University of Bari Medical School Italy

54. Rheumatology Unit, Department of Clinical and Experimental Medicine University of Messina Italy

55. Rheumatology Section, Department of Health Promotion Mother and Child Care, Internal Medicine and Medical Specialties, University Hospital P. Giaccone, University of Palermo Palermo Italy

Abstract

ObjectiveWe aimed to evaluate the clinical usefulness of the systemic score in the prediction of life‐threatening evolution in Still disease. We also aimed to assess the clinical relevance of each component of the systemic score in predicting life‐threatening evolution and to derive patient subsets accordingly.MethodsA multicenter, observational, prospective study was designed including patients included in the Gruppo Italiano Di Ricerca in Reumatologia Clinica e Sperimentale Adult‐Onset Still Disease Study Group and the Autoinflammatory Disease Alliance Network Still Disease Registry. Patients were assessed to see if the variables to derive the systemic score were available. The life‐threatening evolution was defined as mortality, whatever the clinical course, and/or macrophage activation syndrome, a secondary hemophagocytic lymphohistiocytosis associated with a poor prognosis.ResultsA total of 597 patients with Still disease were assessed (mean ± SD age 36.6 ± 17.3 years; male 44.4%). The systemic score, assessed as a continuous variable, significantly predicted the life‐threatening evolution (odds ratio [OR] 1.24; 95% confidence interval [CI] 1.07–1.42; P = 0.004). A systemic score ≥7 also significantly predicted the likelihood of a patient experiencing life‐threatening evolution (OR 3.36; 95% CI 1.81–6.25; P < 0.001). Assessing the clinical relevance of each component of the systemic score, liver involvement (OR 1.68; 95% CI 1.48–2.67; P = 0.031) and lung disease (OR 2.12; 95% CI 1.14–4.49; P = 0.042) both significantly predicted life‐threatening evolution. The clinical characteristics of patients with liver involvement and lung disease were derived, highlighting their relevance in multiorgan disease manifestations.ConclusionThe clinical utility of the systemic score was shown in identifying Still disease at a higher risk of life‐threatening evolution in a large cohort. Furthermore, the clinical relevance of liver involvement and lung disease was highlighted.

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Publisher

Wiley

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