P02-012 - HAIDS in practice of Russian rheumatologist

Results 42 children with HAIDS in age between 2 and 17 years were identified in 3 years. Age of onset ranged from 0 to 16 years, on an average 5.1 years old. The average age at diagnosis was 8.2 years old. The following diseases were identified: Behcet’s disease (BD) 16 children (male/female (M/F) 12/4); Cryopyrin-Associated Periodic Syndromes (CAPS): Muckle-Wells syndrome 4 (M/F 0/4 ); CINCA/NOMID syndrome 2 (M/F 2/0); Familial Mediterranean fever (periodical disease) (FMF) 7 (M/F 1/6); PFAPA syndrome 4 (M/F 2/2), chronic recurrent multifocal (nonbacterial) osteomyelitis (CRMO) 3 (M/F 0/3), hyper-IgD syndrome/mevalonate kinase deficiency 2 (M/F -0/2), undifferentiated HAIDS 4 (M/F = 2/2). Patient’s ethnicity with BD: peoples of the North Caucasus 3, Tatars 4, Uzbeks 1, Azerbaijanians 1, Russians 5, Ukrainians1, ethnic Germans1. Among the patients with FMF: Armenians4, Azerbaijanians2. Russian patients dominated for other nosologies. HLA-B51 antigen was detected in 8 (50%) patients with BD. For all patients with FMF, CAPS and for one patient with hyper-IgD syndrome the diagnoses were confirmed by genotyping.