Table 1 Evolution of the clinical symptoms of PFAPA patients recorded in the registries
Clinical symptoms at diagnosis | JIR-cohort (n = 317) | Eurofever (n = 476) | AID-Net (n = 136) |
|---|---|---|---|
Fever | |||
 < 2009 | - | 130/130 (100%) | - |
 2009 – 2012 | 2/2 (100%) | 211/211 (100%) | 23/23 (100%) |
 2013 – 2016 | 144/144 (100%) | 63/63 (100%) | 67/67 (100%) |
 2017 – 2020 | 171/171 (100%) | 72/72 (100%) | 46/46 (100%) |
Pharyngitis | |||
 < 2009 | - | 97/130 (74.6%) | - |
 2009 – 2012 | 2/2 (100%) | 137/211 (64.9%) | 13/23 (56.5%) |
 2013 – 2016 | 125/144 (86.8%) | 41/63 (65.1%) | 37/67 (55.2%) |
 2017 – 2020 | 133/171 (77.8%) | 48/72 (66.7%) | 23/46 (50%) |
Cervical adenopathy | |||
 < 2009 | - | 98/130 (75.4%) | - |
 2009 – 2012 | 2/2 (100%) | 154/211 (72.9%) | 18/23 (78.2%) |
 2013 – 2016 | 112/144 (77.8%) | 45/63 (71.4%) | 47/67 (70.1%) |
 2017 – 2020 | 113/171 (66.1%)* | 56/72 (77.8%) | 33/46 (71.7%) |
Aphthous stomatitis | |||
 < 2009 | - | 84/130 (64.6%) | - |
 2009 – 2012 | 2/2 (100%) | 91/211 (43.1%) | 11/23 (47.8%) |
 2013 – 2016 | 85/144 (59.0%) | 30/63 (47.6%) | 26/67 (38.8%) |
 2017 – 2020 | 62/171 (36.2%)* | 35/72 (48.6%)* | 20/46 (43.4%) |