Table 3

Overview of clinical and laboratory characteristics observed in patients with A20 haploinsufficiency

FeaturesPatients, n (%)
Mucous membrane16 (100)
Musculoskeletal9 (56)
 Arthritis7 (44)
Gastrointestinal9 (56)
 (Bloody) diarrhoea5 (31)
Recurrent fever8 (50)
Cutaneous8 (50)
Ocular3 (19)
Cardiovascular3 (19)
 Pericarditis2 (13)
Increased acute-phase reactants during disease flare 10/10
Pathergy positive 3/10
Autoantibodies positive 8/14