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. 2019 Nov 6;35(1):117–127. doi: 10.5606/ArchRheumatol.2020.7060

Table 1. Clinical features of patients.

Characteristics Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 Patient 6/sibling of patient 5
Age (year)/sex 4/M 10/F 6/F 8/M 11/M 9/F
Age of initial symptoms 4 month 2 year 5 year 9 month Infancy Infancy
Age of diagnosis (year) 1 9 6 6 10 8
Fever + - - - - -
Arthritis - + + + + +
Dermatitis + - + - - -
Uveitis - - - - - -
Tenosynovitis - + + + + +
Camptodactyly - + + + + +
Kidney - + - - - -
Liver + - - - -  
LAP + - - - - -
Lung - - - - - -
High APR + + - - - +
High ACE - - - - - -
Mutations P268S/V955I M513T/- R702W/SNP8 H343Y/- V955I/M491L M491L
MEFV mutations R761H/- - - E148Q/- E148Q/- -
Previous treatment Steroid, MTX, Steroid, MTX, NSAI, MTX NSAI, MTX, NSAI, MTX NSAI, MTX
ANA, ADA, ETA, TOS ETA, TOS,        
IFN ADA, INF, CAN        
Current treatment LDS, CAN MTX, INF MTX LDS, Siklosporin MTX, INF MTX, INF
LAP: Lymphadenopathy; APR: Acute Phase Reactans; ACE: Angiotensin converting enzyme; MEFV: Mediterranean fever; MTX: Methotrexate; ANA: Anakinra; ADA: Adalimumab; IFN: Infliximab; ETA: Etanercept; TOS: Tosilizumab; NSAI: Non-steroid anti-inflammatory drugs; CAN: Canakinumab; LDS: Low dose steroid.