Table 4 Clinical features of PFAPA patients fulfilling preliminary case definition for SURF and uSAID/SURF patients fulfilling Eurofever PFAPA criteria
| Â | JIR-cohort | Eurofever | AID-net | |||
|---|---|---|---|---|---|---|
PFAPA fulfils SURF (n = 54) | uSAID/SURF fulfils PFAPA (n = 66) | PFAPA fulfils SURF (n = 39) | uSAID/SURF fulfils PFAPA (n = 82) | PFAPA fulfils SURF (n = 16) | uSAID/SURF fulfils PFAPA (n = 21) | |
Age of diagnose, yearsa | 5.11 (0.97 – 22.6) | 5.75 (1.1 – 28.4) | 3.9 (2.1 – 17.6) | 4.35 (2.3 – 26.9) | 4.58 (1.6 – 10.2) | 6.58 (1.7 – 13.2) |
Diagnostic delay, yearsa | 2.87 (0.1 – 17.1) | 1.55 (0.1 – 12.9) | 2.1 (0.8 – 10.9) | 2.38 (0.6 – 11.9) | 2.83 (0.2 – 8.4) | 0.62 (0.2 – 3.4) |
Boys/girls | 27/27 | 35/31 | 21/18 | 39/43 | 5/11 | 9/12 |
Patients < 18 y.o. when diagnosed | 52 (96.3%) | 61 (92.4%) | 39 (100%) | 71 (86.6%) | 16 (100%) | 21 (100%) |
Patients > 18 y.o. when diagnosed | 2 (3.7%) | 5 (7.6%) | 0 | 11 (13.4%) | 0 | 0 |
Enrolled in time- period: | ||||||
 •Under2009 | 0 | 0 | 17 (43.6%) | 15 (18.3%) | 0 | 0 |
 •2009 – 2012 | 0 | 0 | 11 (28.2%) | 28 (34.1%) | 6 (37.5%) | 7 (33.3%) |
 •2013 – 2016 | 17 (31.5%) | 27 (40.9%) | 3 (7.7%) | 15 (18.3%) | 9 (56.2%) | 9 (42.8%) |
 •2017 – 2020 | 37 (68.5%) | 39 (59.1%) | 8 (20.5%) | 24 (29.3%) | 1 (6.3%) | 5 (23.8%) |
 Fever > 38 C | 54 (100%) | 66 (100%) | 39 (100%) | 82 (100%) | 16 (100%) | 21 (100%) |
 -recurrent | 28 (51.8%) | 36 (54.5%) | N/A | N/A | 9 (56.2%) | 15 (71.4%) |
 -continued | 7 (12.9%) | 6 (9.1%) | N/A | N/A | 6 (37.5%) | 6 (28.6%) |
 Duration of the episode, days | 6 (1—11) | 4 (3—6) | 2 (1—11) | 4 (3—6) | 6 (1—10) | 4 (3—5) |
 Regular/ periodic attacks | 52 (100%) | 39 (59.1%) | 22 (56.4%) | 39 (47.6%) | N/A | N/A |
 Irregular/ non periodic attacks | 0 | 21 (31.8%) | 15 (38.5%) | 42 (51.2%) | N/A | N/A |
 Generalized lymphadenopathy | 0 | 1 (1.5%) | 2 (5.1%) | 8 (9.7%) | 0 | 0 |
 Cervical adenopathy | 37 (68.5%) | 31 (46.9%) | 28 (71.7%) | 39 (47.5%) | 9 (56.2%) | 6 (28.6%) |
Muco-cutaneous signs | ||||||
 •Aphthous stomatitis | 11 (20.4%) | 21 (31.8%) | 21 (53.8%) | 28 (34.1%) | 5 (31.2%) | 2 (9.5%) |
 Pharyngitis | 43 (79.6%) | 40 (60.6%) | 14 (35.9%) | 24 (29.2%) | 3 (18.7%) | 6 (28.6%) |
Gastrointestinal involvement | ||||||
 •Abdominal pain/fevers | 20 (37%) | 30 (45.4%) | 10 (25.6%) | 28 (34.1%) | 5 (31.5%) | 7 (33.3%) |
 •Vomitting | 7 (12.9%) | 11 (16.7%) | 5 (12.8%) | 13 (15.8%) | 3 (18.7%) | 0 |
Muscle-skeletal involvement | ||||||
 Artralgia | 16 (29.6%) | 24 (36.4%) | 6 (15.3%) | 17 (20.7%) | 3 (18.7%) | 7 (33.3%) |
 Myalgia | 0 | 1 (1.5%) | 0 | 2 (2.4%) | 0 | 4 (19%) |
 Morning stiffness | 0 | 1 (1.5%) | 0 | 0 | 1 (6.3%) | 0 |
Other manifestations | ||||||
 Headache | 24 (44.4%) | 21 (31.8%) | 17 (43.5%) | 19 (23.1%) | 6 (37.5%) | 5 (23.8%) |
 Hepatomegaly | 1 (1.8%) | 1 (1.5%) | 1 (2.5%) | 1 (1.2%) | 0 | 1 (4.7%) |
 Pleuritis | 0 | 0 | 0 | 0 | 0 | 3 (14.3%) |
 Pericarditis | 0 | 0 | 0 | 0 | 0 | 2 (9.5%) |