Of note, ultrasound revealed little or no inflammation accompanying the PD signal in patient 1 (10 months old) and patient 10 (37 years and 8 months old) even though they had never received anti-inflammatory or rheumatic treatment, whereas patient 2, who is a member of the same family carrying the same NOD2 mutation, exhibited substantial PD tenosynovitis despite receiving an NSAID and methotrexate. Here, NOD2 is linked to tenosynovitis.