Association of Juvenile Idiopathic Arthritis and Digeorge Syndrome; a Case Report

Juvenile idiopathic arthritis (JIA) belongs to a group of arthritis with unknown etiology that occurs in children under 16 years old. Pathogenesis of this disease proposes the role of autoimmune process which is induced by antigens and results in inflammation of synovials and cartilage[1]. DiGeorge syndrome (DGS) or velo-cardio-facial-syndrome (VCFS) is a genetic disorder due to a defect in 22q11.2 chromosome[2]. Patients with 22q11.2 DS usually have characteristic facies including retrognathia or micrognathia, long face, downturned mouth, short philtrum low-set, malformed ears and hypertelorism. Congenital heart defects, either a cleft palate or incompetence of the soft palate, and immune deficiencies are common. Patients may have short stature and occasional instances of growth hormone deficiency[3]. Anomalies related to 22q11 monosomy have a wide range[4]. Renal, pulmonary, gastrointestinal, skeletal, and ophthalmologic abnormalities can also occur. Children and adults with 22q11.2DS have high rates of behavioral, psychiatric, and communication disorders. In children, these include attention-deficit/hyperactivity disorder, anxiety, and affective disorders. Adults have a high rate of psychotic disorders, particularly schizophrenia[3]. Parathyroid dysfunction may cause hypocalcemia and seizures in the neonatal period. Most patients with DGS have a partial form of the syndrome and thymic hypoplasia[5