Comparative Study

. 2024 Sep 1;63(SI2):SI122-SI128.

doi: 10.1093/rheumatology/kead693.

Comparison of EULAR/PRINTO/PReS Ankara 2008 and 2022 ACR/EULAR classification criteria for granulomatosis with polyangiitis in children

Ummusen Kaya Akca¹, Ezgi Deniz Batu¹, Marija Jelusic², Marta Calatroni^{3

4}, Reima Bakry⁵, Marijan Frkovic², Nikol Vinšová⁶, Reinan T Campos⁷, AnnaCarin Horne⁸, Sengul Caglayan⁹, Augusto Vaglio¹⁰, Gabriella Moroni^{3

4}, Giacomo Emmi¹¹, Gian Marco Ghiggeri¹², Oya Koker¹³, Renato Alberto Sinico⁴, Susan Kim¹⁴, Alenka Gagro¹⁵, Caterina Matucci-Cerinic^{16

17}, Elif Çomak¹⁸, Zahide Ekici Tekin¹⁹, Elif Arslanoglu Aydin²⁰, Merav Heshin-Bekenstein²¹, Banu Celikel Acar¹⁹, Marco Gattorno¹⁷, Sema Akman¹⁸, Betul Sozeri⁹, Karin Palmblad⁸, Sulaiman M Al-Mayouf²², Clovis Artur Silva⁷, Pavla Doležalová⁶, Peter A Merkel²³, Seza Ozen¹

Affiliations

¹ Department of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
² Department of Pediatrics, Division of Clinical Immunology, Rheumatology and Allergology, University of Zagreb School of Medicine, Centre of Reference for Paediatric and Adolescent Rheumatology of Ministry of Health of the Republic Croatia, University Hospital Centre Zagreb, Zagreb, Croatia.
³ Department of Biomedical Sciences, Humanitas University, Milan, Italy.
⁴ Nephrology and Dialysis Division, IRCCS Humanitas Research Hospital, Milan, Italy.
⁵ Pediatric Rheumatology, East Jeddah Hospital, Jeddah, Saudi Arabia.
⁶ Centre for Paediatric Rheumatology and Autoinflammatory Diseases, Department of Paediatrics and Inherited Metabolic Disorders, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic.
⁷ Pediatric Rheumatology Unit, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, São Paulo, Brazil.
⁸ Department of Paediatric Rheumatology, Astrid Lindgren Children's Hospital, Stockholm, Sweden.
⁹ Department of Pediatric Rheumatology, Umraniye Training and Research Hospital, Istanbul, Turkey.
¹⁰ Nephrology and Dialysis Unit, Meyer Children's Hospital, Florence, Italy and Department of Biomedical, Experimental and Clinical Sciences, University of Florence, Italy.
¹¹ Department of Experimental and Clinical Medicine, University of Florence, Italy and Internal Interdisciplinary Medicine Unit, Careggi University Hospital, Florence, Italy.
¹² Division of Nephrology, Dialysis, Transplantation, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
¹³ Department of Pediatric Rheumatology, Marmara University Medical School, Istanbul, Turkey.
¹⁴ Division of Pediatric Rheumatology, University of California, San Francisco, California, USA.
¹⁵ Children's Hospital Zagreb, Zagreb, Croatia, School of Medicine, University of Zagreb, Zagreb, Croatia, and Faculty of Medicine, Josip Juraj Strossmayer, Josipa Huttlera 4, University of Osijek, Osijek, Croatia.
¹⁶ Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, Italy.
¹⁷ UOC Rheumatology and Autoinflammatory diseases, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
¹⁸ Pediatric Nephrology and Rheumatology, Faculty of Medicine, Akdeniz University, Antalya, Turkey.
¹⁹ Department of Pediatric Rheumatology, Ankara City Hospital, Ankara, Turkey.
²⁰ Department of Pediatric Rheumatology, Dr. Sami Ulus Maternity and Child Health and Diseases Training and Research Hospital, Ankara, Turkey.
²¹ Pediatric Rheumatology Service, Dana Dwek Children's Hospital, Tel Aviv Medical Center, Tel Aviv University, Tel Aviv, Israel.
²² Division of Rheumatology, Department of Pediatrics, King Faisal Specialist Hospital and Research Center, College of Medicine, Al Faisal University, Riyadh, Saudi Arabia.
²³ Division of Rheumatology, Department of Medicine; Division of Epidemiology, Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, PA, USA.

PMID: 38135503
DOI: 10.1093/rheumatology/kead693

Comparative Study

Comparison of EULAR/PRINTO/PReS Ankara 2008 and 2022 ACR/EULAR classification criteria for granulomatosis with polyangiitis in children

Ummusen Kaya Akca et al. Rheumatology (Oxford). 2024.

. 2024 Sep 1;63(SI2):SI122-SI128.

doi: 10.1093/rheumatology/kead693.

Authors

Affiliations

¹ Department of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
² Department of Pediatrics, Division of Clinical Immunology, Rheumatology and Allergology, University of Zagreb School of Medicine, Centre of Reference for Paediatric and Adolescent Rheumatology of Ministry of Health of the Republic Croatia, University Hospital Centre Zagreb, Zagreb, Croatia.
³ Department of Biomedical Sciences, Humanitas University, Milan, Italy.
⁴ Nephrology and Dialysis Division, IRCCS Humanitas Research Hospital, Milan, Italy.
⁵ Pediatric Rheumatology, East Jeddah Hospital, Jeddah, Saudi Arabia.
⁶ Centre for Paediatric Rheumatology and Autoinflammatory Diseases, Department of Paediatrics and Inherited Metabolic Disorders, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic.
⁷ Pediatric Rheumatology Unit, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, São Paulo, Brazil.
⁸ Department of Paediatric Rheumatology, Astrid Lindgren Children's Hospital, Stockholm, Sweden.
⁹ Department of Pediatric Rheumatology, Umraniye Training and Research Hospital, Istanbul, Turkey.
¹⁰ Nephrology and Dialysis Unit, Meyer Children's Hospital, Florence, Italy and Department of Biomedical, Experimental and Clinical Sciences, University of Florence, Italy.
¹¹ Department of Experimental and Clinical Medicine, University of Florence, Italy and Internal Interdisciplinary Medicine Unit, Careggi University Hospital, Florence, Italy.
¹² Division of Nephrology, Dialysis, Transplantation, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
¹³ Department of Pediatric Rheumatology, Marmara University Medical School, Istanbul, Turkey.
¹⁴ Division of Pediatric Rheumatology, University of California, San Francisco, California, USA.
¹⁵ Children's Hospital Zagreb, Zagreb, Croatia, School of Medicine, University of Zagreb, Zagreb, Croatia, and Faculty of Medicine, Josip Juraj Strossmayer, Josipa Huttlera 4, University of Osijek, Osijek, Croatia.
¹⁶ Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, Italy.
¹⁷ UOC Rheumatology and Autoinflammatory diseases, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
¹⁸ Pediatric Nephrology and Rheumatology, Faculty of Medicine, Akdeniz University, Antalya, Turkey.
¹⁹ Department of Pediatric Rheumatology, Ankara City Hospital, Ankara, Turkey.
²⁰ Department of Pediatric Rheumatology, Dr. Sami Ulus Maternity and Child Health and Diseases Training and Research Hospital, Ankara, Turkey.
²¹ Pediatric Rheumatology Service, Dana Dwek Children's Hospital, Tel Aviv Medical Center, Tel Aviv University, Tel Aviv, Israel.
²² Division of Rheumatology, Department of Pediatrics, King Faisal Specialist Hospital and Research Center, College of Medicine, Al Faisal University, Riyadh, Saudi Arabia.
²³ Division of Rheumatology, Department of Medicine; Division of Epidemiology, Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, PA, USA.

PMID: 38135503
DOI: 10.1093/rheumatology/kead693

Abstract

Objective: Granulomatosis with polyangiitis (GPA) is an ANCA-associated vasculitis. The 2022 ACR/EULAR-endorsed classification criteria for GPA was derived using data only from adult patients. We aimed to assess the performance of the ACR/EULAR classification criteria for GPA in paediatric patients and compare it with the EULAR/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS)-endorsed Ankara 2008 criteria for GPA.

Methods: Retrospective data of paediatric patients with GPA in 20 centres from 9 countries were evaluated. The diagnosis of GPA was made according to the expert opinion. The sensitivity, specificity, positive predictive value, and negative predictive value of the criteria sets were evaluated.

Results: The study included 77 patients with GPA and 108 controls [IgA vasculitis (n = 44), Takayasu's arteritis (n = 20), microscopic polyangiitis (n = 16), polyarteritis nodosa (n = 14), Behçet's disease (n = 12), eosinophilic granulomatosis with polyangiitis (n = 1) and Cogan's syndrome (n = 1)] with a median age of 17.8 and 15.2 years, respectively. Among patients with GPA, constitutional symptoms (85.7%) and ENT involvement (79.2%) were the most common presentations. In the GPA group, 73 patients fulfilled the Ankara 2008 criteria and 69 the ACR/EULAR classification criteria. Sensitivities of the Ankara 2008 criteria and the ACR/EULAR classification criteria were 94.8% and 89.6%, while specificities were 95.3% and 96.3%, respectively. No significant difference was found between sensitivities and specificities of both classification criteria (P = 0.229 and P = 0.733, respectively).

Conclusion: In children, both the ACR/EULAR and EULAR/PRINTO/PReS Ankara 2008 classification criteria for GPA perform well and similarly.

Keywords: ANCA-associated vasculitis; classification criteria; granulomatosis with polyangiitis.

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Comparison of EULAR/PRINTO/PReS Ankara 2008 and 2022 ACR/EULAR classification criteria for granulomatosis with polyangiitis in children

Affiliations

Comparison of EULAR/PRINTO/PReS Ankara 2008 and 2022 ACR/EULAR classification criteria for granulomatosis with polyangiitis in children

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