Table 1

Demographic characteristics of patients enrolled in the registry in the first 18 months

DiseasePatient numberM/FAge at enrollment in years. Median (range)Children*/adults at enrollmentWestern EuropeEastern EuropeEastern – southern MediterreanOthers
FMF554268/28611 (1–86)456/98297421439
TRAPS199101/9823 (2–77)85/114192421
CAPS15279/7315 (0–77)91/611314314
MKD10447/5714 (1–60)65/3994415
Blau2513/1213 (3–51)18/7150010
PAPA73/418 (4–30)3/46100
FCAS265/150 (13–79)2/44002
DIRA20/2 5 (1–9)2/02000
CRMO24588/15714 (1–61)204/412284013
PFAPA361208/153 6 (0–37)352/922166695
Behçet6527/3815 (5–25)47/1852742
Undefined PF16077/8314 (2–71)99/611461211
  • * age <18 years.

  • CAPS, diseases from the spectrum of cryopyrin associated periodic fever syndromes; CRMO, chronic recurrent multifocal osteomyelitis; DIRA, deficiency of the interleukin-1-receptor antagonist; F, female; FCAS2, familial cold autoinflammatory syndrome 2; FMF, familial Mediterranean fever; M, male; MKD, mevalonate-kinase deficiency; PAPA, pyogenic sterile arthritis, pyoderma gangrenosum and acne syndrome; PF, periodic fever; PFAPA, periodic fever syndrome with aphthous stomatitis, pharyngitis and cervical adenopathy; TRAPS, tumour necrosis factor receptor associated periodic syndrome.