PRINTO - Paediatric Rheumatology INternational Trials Organization

PRINTO ongoing project details

Eurofever

The Eurofever project was promoted in 2008 by the work group of autoinflammatory diseases of the Paediatric Rheumatology European Society (PRES) and was supported by the Executive Agency for Health and Consumers (EAHC).

The Eurofever project has established a strong cooperation with the AIDA, the international registry for adult autoinflammatory diseases, PI Dr Luca Cantarini

At this link you can find the Diagnostic score for periodic fevers

The main objective of the project has been the creation of a registry of autoinflammatory diseases.

A recent amendment has been approved in order to:

-add new autoinflammatory diseases according to the new taxonomy (Ben Chetrit et al Ann Rheum Dis 2018)

- add new laboratory results and symptoms in the Case Report Forms

- update consent forms according to the current GDPR

Below is the list of the 32 conditions collected as of today by the Eurofever Registry.

Monogenic autoinflammatory diseases

1.           Blau's disease/Early onset Sarcoidosis

2.           NLRP3-associated autoinflammatory diseases (NLRP3-AID: CINCA/ Muckle Wells/ FCAS)

3.           Deficiency of IL-1 receptor antagonist (DIRA)

4.           Familial Mediterranean Fever (FMF)

5.           Mevalonate kinase Deficiency (MKD or Hyper IgD syndrome)

6.           NLRP12-related disease

7.           PSTPIP1-associated Pyoderma gangrenosum, acne, pyogenic arthritis syndrome (PAPA)

8.           Periodic fever associated to TNFRSF11A (TRAPS11)

9.           TNF receptor associated periodic syndrome (TRAPS)

10.         Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome/proteasome-associated autoinflammatory syndromes (CANDLE/PRAAS)

11.         Deficiency of the IL-3 6receptor antagonist (DITRA syndrome)

12.         Majeed syndrome

13.         Deficiency of Adenosine Deaminase 2 (DADA2)

14.         STING-associated vasculopathy with onset in infancy (SAVI)

15.         CARD14 mediated psoriasis (CAMPS)

16.         IL-10 deficiency-associated Inflammatory bowel disease

17.         Autoinflammation with infantile enterocolitis (NLRC4 – AIFEC)

18.         Autoinflammation, Panniculitis, And Dermatosis Syndrome (OTULIN – AIPDS)

19.         PLCG2-associated antibody deficiency and immune dysregulation (PLAID)

20.         Autoinflammation with arthritis and dyskeratosis

21.         LACC1-associated familial arthritis

22.         Familial acne inversa (Pyoderma gangrenosum, Acne and Suppurative Hidradenitis, PASH)

23.         TNFAIP3-associated autoinflammatory syndrome, familial, behcet-like (AISBL)

24.         Autoimmune interstitial lung, joint and kidney disease (COPA)

Multifactorial auto inflammatory diseases

25.         Behcet's disease

26.         Chronic non bacterial osteomyelitis/osteitis (CNO or Chronic recurrent multifocal osteomyelitis CRMO)

27.         Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA or Marshall's syndrome)

28.         Schnitzler syndrome

29.         Recurrent idiopathic pericarditis

30.         Systemic onset juvenile idiopathic arthritis

31.         Undefined Autoinflammatory disease

32.         Systemic undefined recurrent fever (SURF)

The PI Dr. Marco Gattorno thanks all the 120 centers in 43 countries that collected information of more than 4000 patients with Autoinflammatory diseases.

Several researchers involved in this project are members of the European Reference Network for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases -Project ID No 739543.

We are more than happy to receive any proposal for possible studies based on the Eurofever data! You may send your proposal to printo@gaslini.org and we will submit it to the Steering Committee (M. Gattorno, J. Frenkel, S. Ozen, N. Ruperto, H. Lachmann).

Mastrolia MV, Matucci-Cerinic C, Ozen S, Kasapcopur O, Gaggiano C, Koné-Paut I, Cantarini L, Dusser P, Kaya-Akça Ü, Yildiz M, Brunner J, Filocamo G, Gallizzi R, Insalaco A, Pastore S, Rigante D, Sanchez-Manubens J, Tsitsami E, Ruperto N, Gattorno M, Simonini G
Thrombotic manifestations in pediatric Behcet syndrome: A multicenter comparative study from the EUROFEVER registry
Semin Arthritis Rheum June 2024, 66:152454
Year: 2024 -

Vyzhga Y, Frenkel J, Insalaco A, Anton J, Koné-Paut I, Legger GE, Fabio G, Cattalini M, Kamphuis S, Hachulla E, Krause K, Ekinci Z, Sanchez-Manubens J, Van den Berg JM, Mora CH, Brinkman D, Labrador E, Potjewijd J, Carlini L, Bustaffa M, Caorsi R, Ruperto N, Gattorno M; for Eurofever the Paediatric Rheumatology International Trials Organisation (PRINTO). Monitoring of Adverse Events and Safety in Autoinflammatory Diseases: Real-Life Data from the Eurofever Registry
Monitoring of Adverse Events and Safety in Autoinflammatory Diseases: Real-Life Data from the Eurofever Registry
J Clin Immunol 2024 May 17;44(5):119
Year: 2024 -

Y. Vyzhga, V. Hentgen, R. Caorsi, H. Wittkowski, M. Hofer, N. Ruperto, E. Lainka, K. Theodoropoulou, D. Foell, E. Mosci, M. Gattorno
Breaking down the fences among registries on autoinflammatory diseases: the E-Merge project
Orphanet J Rare Dis 2023 Jul 17;18(1):191
Year: 2023 -

Matucci-Cerinic C, Helene P, Sulaiman AM, Brogan PA, Cantarini L, Gul A, Kasapcopur O, Kuemmerle-Deschner J, Ozen S, Saadoun D, Shahram F, Bovis F, Eugenia M, Ruperto N, Gattorno M, Kone-Paut I; Eurofever for the Paediatric Rheumatology International Trials Organisation (PRINTO)
Validation of the PEDiatric Behçet's Disease classification criteria: an evidence-based approach.
Rheumatology (Oxford) [Epub ahead of print]
Year: 2023 -

Gerritsma AM, Sutera D, Cantarini L, Cattalini M, Lachmann HJ, Minden K, Jansson AF, Touitou I, Bustaffa M, Antón J, Insalaco A, Moreno E, Sanchez-Manubens J, Ruperto N, Frenkel J, Gattorno M
TNFRSF1A-pR92Q variant identifies a subset of patients more similar to systemic undifferentiated recurrent fever than TNF receptor-associated periodic syndrome
Clin Exp Rheumatol. Clin Exp Rheumatol. 2023 Oct;41(10):1998-2007
Year: 2023 -

Bustaffa M, Koné-Paut I, Ozen S, Amaryan G, Papadopoulou-Alataki E, Gallizzi R, Carrabba M, Aviel YB, Cantarini L, Alessio M, Anton J, Obici L, Gok F, Batu ED, Moreno E, Brogan P, Trachana M, Simonini G, Rigante D, Uziel Y, Insalaco A, Maggio MC, Ruperto N, Gattorno M, Semerano LR, for the Pediatric Rheumatology International Trials Organization (PRINTO).
The impact of the Eurofever criteria and the new InFevers MEFV classification in real life: Results from a large international FMF cohort.
Semin Arthritis Rheum 2022; Feb; 52:151957
Year: 2022 -

Bustaffa M, Mazza F, Sutera D, Carrabba MD, Alessio M, Cantarini L, Obici L, Rigante D, Maggio MC, Insalaco A, Simonini G, Cattalini M, Conti G, Olivieri AN, Barone P, Miniaci A, Moressa V, Magnolia MG, Breda L, Montin D, Spagnolo A, Fabio G, Orlando F, Gaggiano C, Mussinelli R, Capozio G, Celani C, Marrani E, Ricci F, Calzatini F, Lancieri M, Ruperto N, Gattorno MM, Gallizzi R, for the Eurofever Registry and for the Pediatric Rheumatology International Trials Organization (PRINTO)
Persistence of disease flares is associated to an inadequate colchicine dose in Familial Mediterranean Fever: a national multicentre longitudinal study.
J Allergy Clin Immunol Pract 2021 Aug;9(8):3218-3220.e1
Year: 2021 -

Papa R, Lane T, Minden K, Touitou I, Cantarini L, Cattalini M, Obici L, Jansson AF, Belot A, Frenkel J, Anton J, Wolska-Kusnierz B, Berendes R, Remesal A, Jelusic M, Hoppenreijs E, Espada G, Nikishina I, Maggio MC, Bovis F, Masini M, Youngstein T, Rezk T, Papadopoulou C, Brogan PA, Hawkins PN, Woo P, Ruperto N, Gattorno M, Lachmann HJ, for the Pediatric Rheumatology International Trials Organization (PRINTO) and the EUROTRAPS and the Eurofever Project.
INSAID variant classification and Eurofever criteria guide optimal treatment strategy in patients with TRAPS: data from the Eurofever registry.
J Allergy Clin Immunol Pract 2021;9(2):783-791
Year: 2021 -

Gattorno M, Hofer M, Vanoni F, Federici S, Cantarini L, Ruperto N for the Paediatric Rheumatology International Trials Organisation (PRINTO) and the Eurofever Registry
Response to: 'Do we need the PFAPA syndrome in adults with non-monogenic periodic fevers?' by Fayand et al
Ann Rheum Dis 2020 Feb 7;annrheumdis-2019-216862
Year: 2020 -

Gattorno M, Hofer M, Federici S, Vanoni F, Ruperto N for the Paediatric Rheumatology International Trials Organisation (PRINTO) and the Eurofever Registry
Some clarifications on the new classification criteria for recurrent fevers
Semin Arthritis Rheum 2020 Dec;50(6):1550-1551
Year: 2020 -

Gattorno M, Hofer M, Federici S, Vanoni F, Bovis F, Aksentijevich I, Anton J, Arostegui JI, Barron K, Ben-Cherit E, Brogan PA, Cantarini L, Ceccherini I, De Benedetti F, Dedeoglu F, Demirkaya E, Frenkel J, Goldbach-Mansky R, Gul A, Hentgen V, Hoffman H, Kallinich T, Kone-Paut I, Kuemmerle-Deschner J, Lachmann HJ, Laxer RM, Livneh A, Obici L, Ozen S, Rowczenio D, Russo R, Shinar Y, Simon A, Toplak N, Touitou I, Uziel Y, van Gijn M, Foell D, Garassino C, Kastner D, Martini A, Sormani MP, Ruperto N, for the Eurofever Registry and the Paediatric Rheumatology International Trials Organisation (PRINTO)
Classification criteria for autoinflammatory recurrent fevers
Ann Rheum Dis 2019;78:1025–1032
Year: 2019 -

Ter Haar NM, Eijkelboom C, Cantarini L, Papa R, Brogan PA, Kone-Paut I, Modesto C, Hofer M, Iagaru N, Fingerhutová S, Insalaco A, Licciardi F, Uziel Y, Jelusic M, Nikishina I, Nielsen S, Papadopoulou-Alataki E, Olivieri AN, Cimaz R, Susic G, Stanevica V, van Gijn M, Vitale A, Ruperto N, Frenkel J, Gattorno M; Eurofever registry and the Pediatric Rheumatology International Trial Organization (PRINTO)
Clinical characteristics and genetic analyses of 187 patients with undefined autoinflammatory diseases
Ann Rheum Dis 2019 Oct;78(10):1405-1411
Year: 2019 -

Gallizzi R, Pidone C, Cantarini L, Finetti M, Cattalini M, Filocamo G, Insalaco A, Rigante D, Consolini R, Maggio MC, Civino A, Martino S, Olivieri AN, Fabio G, Pastore S, Mauro A, Sutera D, Trimarchi G, Ruperto N, Gattorno M, Cimaz R; for Eurofever and the Paediatric Rheumatology International Trials Organisation (PRINTO)
A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry.
Pediatr Rheumatol Online J 2018;Apr 23;16(1):29
Year: 2018 -

Ben-Chetrit E, Gattorno M, Gul A, Kastner DL, Lachmann HJ, Touitou I, Ruperto N for the Paediatric Rheumatology International Trials Organisation (PRINTO) and the AIDs Delphi study participants
Consensus proposal for taxonomy and definition of the autoinflammatory diseases (AIDs): a Delphi study
Ann Rheum Dis 2018;77:1558–1565
Year: 2018 -

Federici S, Vanoni F, Ben-Chetrit E, Cantarini L, Frenkel J, Goldbach-Mansky R, Gul A, Hoffman H, Koné-Paut I, Kuemmerle-Deschner J, Lachmann HJ, Martini A, Obici L, Ozen S, Simon A, Hofer M, Ruperto N, Gattorno M; for Eurofever and the Pediatric Rheumatology International Trials Organization (PRINTO)
An International Delphi Survey for the Definition of New Classification Criteria for Familial Mediterranean Fever, Mevalonate Kinase Deficiency, TNF Receptor-associated Periodic Fever Syndromes, and Cryopyrin-associated Periodic Syndrome
J Rheumatol 2019 Apr;46(4):429-436
Year: 2018 -

Vanoni F, Federici S, Antón J, Barron KS, Brogan P, De Benedetti F, Dedeoglu F, Demirkaya E, Hentgen V, Kallinich T, Laxer R, Russo R, Toplak N, Uziel Y, Martini A, Ruperto N, Gattorno M, Hofer M; for Eurofever and the Paediatric Rheumatology International Trials Organisation (PRINTO)
An international delphi survey for thedefinition of the variables for thedevelopment of new classification criteriafor periodic fever aphtous stomatitispharingitis cervical adenitis (PFAPA)
Pediatr Rheumatol Online J 2018 Apr 18;16(1):27
Year: 2018 -

Papa R, Doglio M, Lachmann HJ, Ozen S, Frenkel J, Simon A, Neven B, Kuemmerle-Deschner J, Ozgodan H, Caorsi R, Federici S, Finetti M, Trachana M, Brunner J, Bezrodnik L, Pinedo Gago MC, Maggio MC, Tsitsami E, Al Suwairi W, Espada G, Shcherbina A, Aksu G, Ruperto N, Martini A, Ceccherini I, Gattorno M, for the Paediatric Rheumatology International Trials Organisation (PRINTO) and the Eurofever Project.
A web-based collection of genotype-phenotype associations in hereditary recurrent fevers from the Eurofever registry.
Orphanet J Rare Dis 2017 Oct 18;12(1):167
Year: 2017 -

Demirkaya E, Saglam C, Turker T, Koné-Paut I, Woo P, Doglio M, Amaryan G, Frenkel J, Uziel Y, Insalaco A, Cantarini L, Hofer M, Boiu S, Duzova A, Modesto C, Bryant A, Rigante D, Papadopoulou-Alataki E, Guillaume-Czitrom S, Kuemmerle-Deschner J, Neven B, Lachmann H, Martini A, Ruperto N, Gattorno M, Ozen S for the Paediatric Rheumatology International Trials Organisations (PRINTO) and Eurofever Project.
Performance of Different Diagnostic Criteria for Familial Mediterranean Fever in Children with Periodic Fevers: Results from a Multicenter International Registry.
J Rheumatol 2016;43:154-160.
Year: 2016 -

Ter Haar NM, Jeyaratnam J, Lachmann HJ, Simon A, Brogan PA, Doglio M, Cattalini M, Anton J, Modesto C, Quartier P, Hoppenreijs E, Martino S, Insalaco A, Cantarini L, Lepore L, Alessio M, Calvo Penades I, Boros C, Consolini R, Rigante D, Russo R, Pachlopnik Schmid J, Lane T, Martini A, Ruperto N, Frenkel J, Gattorno M; Paediatric Rheumatology International Trials Organisation (PRINTO) and Eurofever Project.
The phenotype and genotype of mevalonate kinase deficiency: a series of 114 cases from the Eurofever Registry
Arthritis Rheum 2016;68(11):2795-2805
Year: 2016 -

Levy R, Gérard L, Kuemmerle-Deschner J, Lachmann HJ, Koné-Paut I, Cantarini L, Woo P, Naselli A, Bader-Meunier B, Insalaco A, Al-Mayouf SM, Ozen S, Hofer M, Frenkel J, Modesto C, Nikishina I, Schwarz T, Martino S, Meini A, Quartier P, Martini A, Ruperto N, Neven B, Gattorno M for the Paediatric Rheumatology International Trials Organisation (PRINTO) and Eurofever.
Phenotypic and genotypic characteristics of cryopyrin-associated periodic syndrome: a series of 136 patients from the Eurofever Registry.
Ann Rheum Dis 2015;74:2043–2049.
Year: 2015 -

Federici S, Sormani , Ozen S, Lachmann HJ, Amaryan G, Woo P,6 Koné-Paut I, Dewarrat N, Cantarini L, Insalaco A, Uziel Y, Rigante D, Quartier P, Demirkaya E, Herlin T, Meini A, Fabio G, Kallinich T, Martino S, Butbul AY, Olivieri A, Kuemmerle-Deschner J, Neven B, Simon A, Ozdogan H, Touitou I, Frenkel J, Hofer M, Martini A, Ruperto N, Gattorno M for the Paediatric Rheumatology International Trials Organisation (PRINTO) and Eurofever Project.
Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers.
Ann Rheum Dis 2015;74(5):799-805
Year: 2015 -

Lachmann HJ, Papa R, Gerhold K, Obici L, Touitou I, Cantarini L, Frenkel J, Anton J, Kone-Paut I, Cattalini M, Bader-Meunier B, Insalaco A, Hentgen V, Merino R, Modesto C, Toplak N, Berendes R, Ozen S, Cimaz R, Jansson A, Brogan P, Hawkins PN, Ruperto N, Martini A, Woo P, Gattorno M for the Paediatric Rheumatology International Trials Organisation (PRINTO); the Eurotraps and the Eurofever Project.
The phenotype of TNF-receptor associated autoinflammatory syndrome (TRAPS) at presentation: a series of 158 cases from the EUROFEVER/EUROTRAPS international registry
Ann Rheum Dis 2014;73:2160-2167.
Year: 2014 -

Piram M, Koné-Paut I, Lachmann HJ, Frenkel J, Ozen S, Kuemmerle-Deschner J, Stojanov S, Simon A, Finetti M, Sormani MP, Martini A, Gattorno M, Ruperto N on the behalf of EUROFEVER;EUROTRAPS and the Paediatric Rheumatology International Trials Organisation (PRINTO) networks.
Validation of the auto-inflammatory disease activity index (AIDAI) for hereditary recurrent fever syndromes.
Ann Rheum Dis 2014;73:2168-2173
Year: 2014 -

Ozen S, Demirkaya E, Amaryan G, Koné-Paut I, Polat A, Woo P, Uziel Y, Modesto C, Finetti M, Quartier P, Papadopoulou-Alataki E, Al-Mayouf SM, Fabio G, Gallizzi R, Cantarini L, Frenkel J, Nielsen S, Hofer M, Insalaco A, Acikel C, Ozdogan H, Martini A, Ruperto N, Gattorno M; for the Paediatric Rheumatology International Trials Organisation (PRINTO) and the Eurofever Project.
Results from a multicentre international registry of familial Mediterranean fever: impact of environment on the expression of a monogenic disease in children.
Ann Rheum Dis 2014;73:662–667
Year: 2014 -

ter Haar NM , Lachmann HJ, Ozen S, Woo P, Uziel Y, Modesto C, Koné-Paut I, Cantarini L, Insalaco A, Neven B, Hofer M, Rigante D, Al-Mayouf SM, Touitou I, Gallizzi R, Papadopoulou-Alataki E, Martino S, Kuemmerle-Deschner JB, Obici L, Iagaru N, Simon A, Nielsen S, Martini A, Ruperto N, Gattorno M, Frenkel J for the Paediatric Rheumatology International Trials Organisation (PRINTO) and the Eurofever/Eurotraps Projects.
Treatment of autoinflammatory diseases: results from the Eurofever registry and a literature review.
Ann Rheum Dis 2013;72:678-685
Year: 2013 -

Toplak N, Frenkel J, Ozen S, Lachmann HJ, Woo P, Koné-Paut I, De Benedetti F, Neven B, HoferM, Dolezalova P, Kümmerle-Deschner J, Touitou I, Hentgen V, Simon A, Girschick H, Rose C, Wouters C, Vesely R, Arostegui J, Stojanov S, Ozgodan H, Martini A, Ruperto N, Gattorno M for the Paediatric Rheumatology International Trials Organisation (PRINTO).
An International registry on Autoinflamatory diseases: the Eurofever experience.
Ann Rheum Dis 2012;71:1177-1182
Year: 2012 -

Ozen S, Frenkel J, Ruperto N, Gattorno M on behalf of the Eurofever Project
The Eurofever project: towards better care for autoinflammatory diseases
Eur J Pediatr 2011;170:445-452
Year: 2011 -

Toplak N, Dolezalovà P, Constantin T, Sedivà A, Pašiæ S, Èižnar P, Wolska-Kuœnierz B, Harjaèek M, Stefan M, Ruperto N, Gattorno M, Avèin T and Eastern/Central European autoinflammatory collaborating group for the Paediatric Rheumatology International Trials Organisation (PRINTO) and Eurofever Project.
Periodic fever syndromes in Eastern and Central European countries: results of a pediatric. multinational survey.
Pediatr Rheumatol Online J 2010;8:29.
Year: 2010 -

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PRINTO ongoing project details

Eurofever

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