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).