Table 3 Epidemiological and clinical data of the patients with uSAID/SURF according to the preliminary case definition for SURF (11)
|  | JIR-cohort (n = 175) | Eurofever (n = 307) | AID-net (n = 79) | |||
|---|---|---|---|---|---|---|
uSAID/SURF fulfil SURF case definition and do not met PFAPA (n = 136) | uSAID/SURF do not met SURF case definition (n = 39) | uSAID/SURF fulfil SURF case definition and do not met PFAPA (n = 230) | uSAID/SURF do not met SURF case definition (n = 77) | uSAID/SURF fulfil SURF case definition and do not met PFAPA (n = 51) | uSAID/SURF do not met SURF case definition (n = 28) | |
Age of diagnose, yearsa | 6.6 (0.9 – 63.4) | 7.2 (2.9 – 29.8) | 6.4 (3.1 – 9.2) | 8.2 (3.6 – 21.3) | 7.15 (1.0—18) | 7.71 (2.41 – 18.7) |
Diagnostic delay, yearsa | 1.99 (0.1 – 47.9) | 2.1 (0.7 – 6.61) | 2.9 (0.6 – 11.2) | 3.2 (1.9 – 7.4) | 0.5 (0 – 17.5) | 1.08 (0.1 – 17.5) |
Boys/girls | 67/69 | 17/22 | 119/111 | 44/33 | 33/18 | 17/11 |
Patients < 18 y.o. when diagnosed | 104 (76.5%) | 35 (89.7%) | 196 (85.2%) | 67 (87%) | 46 (90.2%) | 26 (92.8%) |
Patients > 18 y.o. when diagnosed | 32 (23.5%) | 4 (10.3%) | 34 (14.8%) | 10 (13%) | 5 (9.8%) | 2 (7.1%) |
Enrolled in time-period: | ||||||
 •Under 2009 | 0 | 0 | 54 (23.5%) | 20 (26%) | 0 | 0 |
 •2009 – 2012 | 0 | 0 | 91 (39.5%) | 35 (45.4%) | 26 (51%) | 4 |
 •2013 – 2016 | 46 (33.8%) | 13 (33.3%) | 41 (17.9%) | 11 (14.3%) | 19 (37.2%) | 10 |
 •2017 – 2020 | 90 (66.2%) | 26 (66.7%) | 44 (19.1%) | 11 (14.3%) | 6 (11.8%) | 14 |
 Fever > 38 C | 136 (100%) | 39 (100%) | 230 (100%) | 74 (96.1%) | 51 (100%) | 28 (100%) |
 -recurrent | 83 (61%) | 18 (46.1%) | N/A | N/A | 43 (84.3%) | 20 (71.4%) |
 -continued | 9 (6.6%) | 5 (12.8%) | N/A | N/A | 8 (15.7%) | 5 (17.8%) |
 Duration of the episode, days | 4 (3—6) | 2 (1—8) | 4 (3—6) | 2 (1—10) | 4 (1—20) | 2 (1—10) |
 Regular/ periodic attacks | 59 (43.4%) | 17 (43.6%) | 88 (38.3%) | 31 (40.2%) | N/A | N/A |
 Irregular/ non periodic attacks | 56 (41.2%) | 16 (41%) | 123 (53.5%) | 46 (59.7%) | N/A | N/A |
 Generalized lymphadenopathy | 4 (2.9%) | 1 (2.5%) | 18 (7.8%) | 4 (5.2%) | 2 (3.9%) | 2 (7.1%) |
 Cervical adenopathy | 43 (31.6%) | 11 (28.2%) | 81 (35.2%) | 25 (32.5%) | 10 (19.6%) | 4 (14.3%) |
Muco-cutaneous signs | ||||||
 •Aphthous stomatitis | 32 (23.5%) | 6 (15.4%) | 64 (27.8%) | 17 (22.1%) | 4 (7.8%) | 2 (7.1%) |
 •Rash | 31 (22.8%) | 6 (15.4%) | 48 (21%) | 14 (18.2%) | 27 (53%) | 13 (46.4%) |
 •Periorbital edema | 3 (2.2%) | 0 | 2 (0.1%) | 0 | 2 (3.9%) | 0 |
 Pharyngitis | 40 (29.4%) | 6 (15.4%) | 54 (23.5%) | 10 (13%) | 7 (13.7%) | 0 |
Gastrointestinal involvement | ||||||
 •Abdominal pain/fevers | 63 (46.3%) | 20 (51.3%) | 112 (48.7%) | 38 (49.3%) | 17 (33.3%) | 10 (35.7%) |
 •Diarrhoea | 31 (22.8%) | 8 (20.5%) | 64 (27.8%) | 12 (15.6%) | 7 (13.7%) | 4 (14.3%) |
 •Vomiting | 36 (26.5%) | 14 (35.9%) | 52 (22.6%) | 22 (29.8%) | 3 (5.9%) | 3 (10.7%) |
Muscle-skeletal involvement | ||||||
 Arthritis | 10 (7.3%) | 2 (5.1%) | 13 (5.6%) | 3 (3.9%) | 8 (15.7%) | 5 (17.8%) |
 Arthralgia | 64 (47%) | 33 (84.6%) | 110 (47.8%) | 42 (54.5%) | 20 (39.2%) | 12 (42.8%) |
 Myalgia | 4 (2.9%) | 3 (7.7%) | 24 (10.4%) | 11 (14.2%) | 8 (15.7%) | 5 (17.8%) |
 Morning stiffness | 9 (6.6%) | 1 (2.6%) | 11 (4.7%) | 3 (3.9%) | 5 (9.8%) | 0 |
 Other manifestations |  |  |  |  |  |  |
 Conjunctivitis | 4 (2.9%) | 0 | 6 (2.6%) | 0 | 0 | 0 |
 Chest pain | 16 (11.7%) | 5 (12.8%) | 37 (16.1%) | 11 (14.3%) | 1 (1.9%) | 1 (3.6%) |
 Headache | 41 (30.1%) | 11 (28.2%) | 72 (31.3%) | 17 (22.1%) | 14 (27.4%) | 8 (28.6%) |
 Hepatomegaly | 3 (2.2%) | 0 | 5 (2.2%) | 1 (1.3%) | 2 (3.9%) | 1 (3.6%) |
 Splenomegaly | 2 (1.5%) | 0 | 3 (1.3%) | 0 | 8 (15.6%) | 3 (10.7%) |
 Pleuritis | 3 (2.2%) | 0 | 0 | 0 | 7 (13.7%) | 1 (3.6%) |
 Pericarditis | 6 (4.4%) | 0 | 0 | 0 | 6 (11.7%) | 2 (7.1%) |
Other manifestations | ||||||
 Conjunctivitis | 4 (2.9%) | 0 | 6 (2.6%) | 0 | 0 | 0 |
 Chest pain | 16 (11.7%) | 5 (12.8%) | 37 (16.1%) | 11 (14.3%) | 1 (1.9%) | 1 (3.6%) |
 Headache | 41 (30.1%) | 11 (28.2%) | 72 (31.3%) | 17 (22.1%) | 14 (27.4%) | 8 (28.6%) |
 Hepatomegaly | 3 (2.2%) | 0 | 5 (2.2%) | 1 (1.3%) | 2 (3.9%) | 1 (3.6%) |
 Splenomegaly | 2 (1.5%) | 0 | 3 (1.3%) | 0 | 8 (15.6%) | 3 (10.7%) |
 Pleuritis | 3 (2.2%) | 0 | 0 | 0 | 7 (13.7%) | 1 (3.6%) |
 Pericarditis | 6 (4.4%) | 0 | 0 | 0 | 6 (11.7%) | 2 (7.1%) |