Clinical Features and Genetic Background of the Periodic Fever Syndrome with Aphthous Stomatitis, Pharyngitis, and Adenitis: A Single Center Longitudinal Study of 81 Patients

Table 3

Family history.

Recurrent fever or/and tonsillectomy in first degree relative	50/64 (78%)
Recurrent fever or/and tonsillectomy in first degree relative, one family member affected only	29/64 (45%)
Recurrent fever or/and tonsillectomy in first degree relative, more than one family member affected	21/64 (38%)
Tonsillectomy in first degree relative	36/64 (56%)
Recurrent fever without tonsillectomy in first degree relative	14/64 (22%)
Tonsillectomy in second degree relative only^*	6/64 (9%)
Negative	8/64 (13%)
Unknown	17/81 (21%)

Excluding those with recurrent fever or tonsillectomy in first degree relative.