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A rare disease with many faces: A multicenter registry of IgG4-related disease in children.
Kaya Akca, Ummusen; Kose, Hulya; Kurt, Tuba; Ulu, Kadir; Guliyeva, Vafa; Kilbas, Gulsah; Arslanoglu, Ceyda; Yildirim, Deniz Gezgin; Demir, Selcan; Sahin, Sezgin; Kisaarslan, Aysenur Pac; Kasap Demir, Belde; Sonmez, Hafize Emine; Koker, Oya; Yardimci, Gozde Kubra; Ekici, Mustafa; Kilic, Sara Sebnem; Acar, Banu Celikel; Sozeri, Betul; Aktay Ayaz, Nuray; Yuksel, Selcuk; Bakkaloglu, Sevcan Azime; Kasapcopur, Ozgur; Ayhan, Emine Arzu; Karadag, Omer; Ozen, Seza; Bilginer, Yelda.
Afiliação
  • Kaya Akca U; Department of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
  • Kose H; Department of Pediatric Immunology and Rheumatology, Uludag University Faculty of Medicine, Bursa, Turkey.
  • Kurt T; Department of Pediatric Rheumatology, Ankara City Hospital, University of Health Sciences, Ankara, Turkey.
  • Ulu K; Department of Pediatric Rheumatology, Umraniye Training and Research Hospital, University of Health Sciences, Istanbul, Turkey.
  • Guliyeva V; Department of Pediatric Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Turkey, Istanbul.
  • Kilbas G; Department of Pediatric Rheumatology, Pamukkale University Faculty of Medicine, Denizli, Turkey.
  • Arslanoglu C; Department of Pediatric Rheumatology, Erciyes University Faculty of Medicine, Kayseri, Turkey.
  • Yildirim DG; Department of Pediatric Rheumatology, Gazi University Faculty of Medicine, Ankara, Turkey.
  • Demir S; Department of Pediatric Rheumatology, Erzurum Regional Education and Research Hospital, Erzurum, Turkey.
  • Sahin S; Department of Pediatric Rheumatology, Istanbul University Cerrahpasa Faculty of Medicine, Istanbul, Turkey.
  • Kisaarslan AP; Department of Pediatric Rheumatology, Erciyes University Faculty of Medicine, Kayseri, Turkey.
  • Kasap Demir B; Department of Pediatric Nephrology and Rheumatology, Izmir Katip Celebi University Faculty of Medicine, Izmir, Turkey.
  • Sonmez HE; Department of Pediatric Rheumatology, Kocaeli University Faculty of Medicine, Kocaeli, Turkey.
  • Koker O; Department of Pediatric Rheumatology, Marmara University Faculty of Medicine, Istanbul, Turkey.
  • Yardimci GK; Department of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
  • Ekici M; Department of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
  • Kilic SS; Department of Pediatric Immunology and Rheumatology, Uludag University Faculty of Medicine, Bursa, Turkey.
  • Acar BC; Department of Pediatric Rheumatology, Ankara City Hospital, University of Health Sciences, Ankara, Turkey.
  • Sozeri B; Department of Pediatric Rheumatology, Umraniye Training and Research Hospital, University of Health Sciences, Istanbul, Turkey.
  • Aktay Ayaz N; Department of Pediatric Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Turkey, Istanbul.
  • Yuksel S; Department of Pediatric Rheumatology, Pamukkale University Faculty of Medicine, Denizli, Turkey.
  • Bakkaloglu SA; Department of Pediatric Rheumatology, Gazi University Faculty of Medicine, Ankara, Turkey.
  • Kasapcopur O; Department of Pediatric Rheumatology, Istanbul University Cerrahpasa Faculty of Medicine, Istanbul, Turkey.
  • Ayhan EA; Department of Pathology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
  • Karadag O; Department of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
  • Ozen S; Department of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
  • Bilginer Y; Department of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Article em En | MEDLINE | ID: mdl-39298509
ABSTRACT

OBJECTIVES:

We aimed to report the characteristics of pediatric IgG4-related disease (IgG4-RD) through a multicentre registry, to assess disease clusters, and to evaluate the performances of the 2019 American College of Rheumatology and European League Against Rheumatism (ACR/EULAR) classification criteria and the 2020 revised comprehensive diagnostic (RCD) criteria in this cohort.

METHODS:

Data of IgG4-RD patients in 13 pediatric rheumatology centers were recorded to a web-based registration system. The diagnosis of IgG4-RD was made according to the 2011 comprehensive diagnostic criteria.

RESULTS:

Thirty-five children (19 females and 16 males) with IgG4-RD were enrolled. The median age at diagnosis was 13.3 (25p-75p; 9.9-15.2) years. The most common organ involvement was the eye (n = 21, 60%), followed by lymph nodes (n = 12, 34.3%), musculoskeletal system (n = 12, 34.3%), and neurological system (n = 9, 25.7%). We identified three clusters in our study cohort those with eye involvement (n = 11, 31.4%), those with eye involvement and neurological findings (n = 15, 42.9%), and those with pancreato-hepatobiliary disease and lymph node involvement (n = 9, 25.7%). Serum IgG4 levels were high in 19 out of 28 patients (67.8%). All patients except one received corticosteroid treatment, and azathioprine was the most preferred drug as a steroid-sparing agent. The sensitivities of the 2019 ACR/EULAR classification criteria and the 2020 RCD criteria were 5.7% and 88.5%, respectively.

CONCLUSION:

IgG4-RD has a wide variety of clinical manifestations, however in children the most common presentation was orbital involvement. The 2020 RCD criteria had a better performance whereas the 2019 ACR/EULAR classification criteria performed poorly in pediatric patients.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Idioma: En Revista: Rheumatology (Oxford) Assunto da revista: REUMATOLOGIA Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Turquia País de publicação: Reino Unido

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Idioma: En Revista: Rheumatology (Oxford) Assunto da revista: REUMATOLOGIA Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Turquia País de publicação: Reino Unido