Basic Characteristics of Adults with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy Syndrome in Comparison with the Typical Pediatric Expression of Disease
Table 1
Comparison between adult PFAPA and pediatric PFAPA syndrome regarding the main demographic and clinical characteristics and response to treatment.
Adult PFAPA syndrome
Pediatric PFAPA syndrome
Number of patients
36
268
Gender
18 M/18 F
148 M/120 F
Mean age at diagnosis
30.3 ± 9.3
4.8 ± 2.2
Mean age at disease onset
21.6 ± 10.6
2.4 ± 2
Mean number of febrile episodes per year
8.3 ± 5.2 (16/36 adult patients available)
13.3 ± 9.2
Presence of 3 cardinal signs (aphthous stomatitis, cervical lymphadenitis, and pharyngitis)
17/36
74/268
Combination of 2 among 3 cardinal signs
19/36
124/268
Combination of recurrent fever + only 1 cardinal sign
—
70/268
Response to corticosteroids
Complete response in 28, partial response in 4, and ineffective response in 1
Complete response in 226 and partial response in 2
Response to tonsillectomy
Complete in 1, partial in 2, and ineffective in 13 (tonsillectomies performed during infancy included)
